|Year : 2018 | Volume
| Issue : 1 | Page : 9-17
Multimodality Imaging and Clinical Significance of Congenital Ventricular Outpouchings: Recesses, Diverticula, Aneurysms, Clefts, and Crypts
Alberto Cresti1, Pierpaolo Cannarile1, Elena Aldi2, Marco Solari1, Bruno Sposato3, Luca Franci4, Ugo Limbruno1
1 Department of Cardiology, Misericordia Hospital, Grosseto, Italy
2 Department of Radiology, University of Siena, Siena, Italy
3 Department of Internal Medicine, Misericordia Hospital, Grosseto, Italy
4 Department of Radiology, Misericordia Hospital, Grosseto, Italy
|Date of Web Publication||6-Mar-2018|
Dr. Alberto Cresti
Via Etiopia, 131, Grosseto 58100
Source of Support: None, Conflict of Interest: None
The high spatial resolution of cardiac computed tomography (CT) and cardiac magnetic resonance (CMR) permit the diagnosis of congenital ventricular outpouchings (CVOs), including congenital ventricular diverticula (CVD), congenital ventricular aneurysms (CVA), clefts, and crypts. A unique classification has not been established, and these terms are used interchangeably with confounding terminology. Moreover, their significance is not univocal. A research was performed using PubMed on six subjects: (1) congenital left ventricular outpouchings; (2) congenital ventricular diverticulum; (3) congenital ventricular aneurysm; (4) ventricular clefts; (5) ventricular crypts; and (6) ventricular crevices. Usually, CVOs are small with a preserved contraction and in asymptomatic patients, the clinical relevance may be minimal, although electrocardiographic anomalies are often present. CVA and diverticula may carry an embolic risk and cases of arrhythmia and rupture are described. In the presence of clefts, or crypts a cardiomyopathy should be excluded. A simple classification can be proposed: CVD extend beyond the myocardial wall and fibrous type may be termed CVA, acquired forms should be kept distinct. Clefts, or crypts, are small recesses extending for more than 50% of the ventricular wall but not beyond its margin. The presence of fibrosis may be evaluated by CMR. A multicenter prospective registry would be helpful to investigate potential clinical implications and to exclude dubious forms of hypertrophic cardiomyopathy or ventricular noncompaction. In conclusion, CVOs have been described with different terminologies and classifications. Their significance needs to be interpreted in the clinical setting and with the help of a multimodality imaging, particularly of CMR.
Keywords: Aneurysm, cardiac diverticulum, false aneurysm, myocardial crypt, pseudoaneurysm, ventricular diverticula
|How to cite this article:|
Cresti A, Cannarile P, Aldi E, Solari M, Sposato B, Franci L, Limbruno U. Multimodality Imaging and Clinical Significance of Congenital Ventricular Outpouchings: Recesses, Diverticula, Aneurysms, Clefts, and Crypts. J Cardiovasc Echography 2018;28:9-17
|How to cite this URL:|
Cresti A, Cannarile P, Aldi E, Solari M, Sposato B, Franci L, Limbruno U. Multimodality Imaging and Clinical Significance of Congenital Ventricular Outpouchings: Recesses, Diverticula, Aneurysms, Clefts, and Crypts. J Cardiovasc Echography [serial online] 2018 [cited 2022 Nov 29];28:9-17. Available from: https://www.jcecho.org/text.asp?2018/28/1/9/226686
| Introduction|| |
The high spatial resolution of cardiac computed tomography (CT) and cardiac magnetic resonance (CMR) enable the diagnosis of congenital ventricular recesses and congenital ventricular outpouchings (CVOs), including congenital ventricular diverticula (CVD), congenital ventricular aneurysms (CVA), clefts, and crypts. Nowadays, these anomalies seem to be more frequent than previously thought. The presence of a myocardial “crypt” is reported in up to 6.7% of all the cardiac CT scans.
A unique definition and classification of different types of ventricular outpouchings have not been established yet and the terms diverticula, aneurysm, crypts are often used interchangeably with confounding terminology.
Among CVOs, some Authors include accessory ventricles and chambers, the so-called double-chambered left ventricle (DCLV), while other Authors have introduced the term pseudodiverticulum to define the fibrous subtype. These different terminologies and classifications are confusing, and agreement among experts is warranted as already suggested by several investigators.,,
The clinical relevance of CVOs is a clinical dilemma, especially when electrocardiographic anomalies are present in asymptomatic patients. Which level of physical activity or competitive sport can be prescribed still need an answer.
Performing a review on the diagnostic criteria of all types of CVOs, our aim was to evaluate the definitions that have been used in recent literature to invite an expert consensus for a standardized and comprehensive classification with prognostic implications. A simple classification is proposed.
| Methods|| |
A research was performed using PubMed electronic database on five subjects: (1) congenital left ventricular outpouchings (LVO); (2) congenital ventricular diverticulum; (3) CVA; (4) ventricular clefts; (5) ventricular crypts; and (6) ventricular crevices. The last search was performed on March 2017.
Review articles, case series and relevant isolated case reports with important clinical points were evaluated. All patients with all types of CVOs from prenatal to geriatric age range were investigated. In case of multiple papers published by the same Author, the largest and newest database was evaluated.
We reviewed a total of 110 studies including review articles and case series. Whether all reported cases should be really considered true congenital outpouchings is not always discernible and some papers have not been included in the final analysis. As an example, Chen et al. included in his study acquired cases of ventricular outpouchings. In the review process, the PRISMA guidelines were followed.
The largest case-series from prenatal age to adulthood, about 500 cases, was presented by Ohlow.
| Current Definitions and Classification|| |
Congenital ventricular diverticula
CVD are rare cardiac malformations described for the first time by O'Bryan in 1838. They are characterized by an outpouching of the entire ventricular wall and a corresponding finger-like protrusion from the internal cavity extending outside the epicardium margin and a narrow connection to the cavity.
Depending on the amount of myocardial fibers involved, diverticula may be classified in muscular or fibrous. Muscular diverticula contain all layers of the ventricular myocardium with the myocardial architecture preserved and minimal fibrous tissue. [Table 1] shows the main diagnostic features of muscular and fibrous diverticula are summarized. Muscular forms tend to show a contractile function synchronous with the ventricle. Fibrous diverticula have connective tissue composed of reticulin and muscle fibers are few or absent. Fibrous forms show either an akinetic or dyskinetic contractile function and usually a wider neck. Nam et al. divided CVD in apical and nonapical. The term pseudodiverticulum has been used by some Authors to define the fibrous type of CVD recommending delayed enhancement CMR imaging to differentiate pseudodiverticulum from true diverticulum. In our opinion, this definition adds a further element of terminological confusion and should be avoided.
|Table 1: Differential diagnosis between muscular and fibrous diverticula|
Click here to view
Fibrous diverticula seem to be indistinguishable from congenital aneurysm and carry the same clinical significance. The term fibrous diverticulum is less used in recent literature.
CVD may be isolated or associated to midline thoraco-abdominal congenital abnormalities. CVD as part of a complex malformation syndrome are usually detected in children and have been described in detail by Cantrell et al. in 1958 as part of a pentalogy including (1) median line abdominal wall defects (omphalocele); (2) inferior sternal defects (aplasia and cleft sternum); (3) anterior diaphragmatic defects; (4) diaphragmatic pericardium defects (absence of the inferolateral pericardium); and (5) complex cardiac abnormalities. Cantrell's pentalogy may be underreported as phenotypic expression is often incomplete. However, diverticula without midline abnormalities are increasingly reported and nowadays are by far the most common in the adult population.
CVD are typically single but may be multiple. Diverticula diameter may range from 0.5 to 9 cm. They are most often found in the LV, but all chambers may be affected and rarely may be multiple (“ventricular diverticulosis”).
Although diverticula can theoretically be found in any segment, they are generally reported in the apex and perivalvular area; they have never been found in the ventricular septum.
CVD not associated with congenital malformations, namely, isolated left ventricular diverticulum, represent an underestimated anomaly whose prevalence in the general population is not known. Shauq reported CVD as 0.05% of all congenital heart malformations, but the prevalence varies according to different imaging modalities, ranging from 0.4% in autoptic series, to 0.26% - 0.76 in patients undergoing cardiac ventricular angiography. Aquaro, in a population of patients who underwent CMR, found a prevalence of isolated CVD of 0,75%. Nakazono et al. reported an incidence of left and right ventricular diverticula on 256-slice CT of 3.4% and 0.6%, respectively. [Table 2] shows LVO frequencies reported from different studies are summarized.
|Table 2: Incidence of ventricular outpouchings according to different diagnostic studies|
Click here to view
| Congenital Ventricular Aneurysms|| |
From a histological point of view, VA lack completely the myocardial layer; they also show akinetic or dyskinetic wall with asynchronous or paradoxical contraction and may exhibit multiple necks.
The term CVA has been used when the morphology of the ventricular outpouching resembled an acquired aneurysm, with a wide connection to the ventricular cavity and without midline thoraco-abdominal defects. It has been proposed to use the term aneurysm only for acquired forms of ventricular outpouchings, secondary to a myocardial damage (myocardial infarction, myocarditis, cardiomyopathy, trauma, tuberculosis, connective tissue disease, and ventricular dysplasia) and to define congenital outpouchings diverticula. On the contrary, other Authors proposed to identify CVD only with the muscular types and use the term aneurysms for the fibrous type. CVA are probably the same entity as CVD of the fibrous type.
Myocardial crypts and clefts
Myocardial crypts are narrow, deep invaginations within the myocardium localized predominantly in the basal posterior septum and LV-free wall. They are congenital fissures related to the disarray of the myocardial fibers.
Crypts have been defined as “a discrete approximately V- or U-shaped extension of blood signal, considered on cine viewing to penetrate more than 50% of the thickness of the adjoining compact myocardium in diastole” narrowing or completely occluding in systole, without segmental contractility defects.,
The term “partial crypts” has been proposed to define recesses penetrating 25%–50% of the wall thickness, but they may represent just irregularities due to trabeculations arising from the endocardial contour without any clinical significance.
Myocardial crypts have been described for the first time at autopsy in patients with hypertrophic cardiomyopathy (HCM). They are generally localized between the muscle bundles of the junctional regions between the ventricles. The crossing and interdigitation of myocytes in this location may predispose to myocardial splitting.
The term “myocardial cleft” has been used especially in the context of HCM, to define narrow invaginations within the left ventricular myocardium.
CMR has enabled in vivo detection of clefts in carriers of HCM mutations and healthy volunteers. Among 16 HCM mutation carriers, who had not yet developed frank hypertrophy, Germans et al. found similar recesses in 13 (81%) and called them crypts. The Author postulated that crypts may have a potential pathological significance as they could precede the phenotypic evidence of HCM. In contrast, Petryka et al. reported a prevalence of 15.6% in HCM, 13.6% of hypertensive patients and 6% of healthy volunteers, suggesting them to be, in most of the cases, only “innocent bystanders.”
A similar incidence has been reported by Erol et al. 6.7% ventricular clefts among 2093 patients undergoing cardiac CT scan while Ozturk et al., in a similar population, found an incidence of 3.05%.
In unselected patients undergoing a cardiac CT scan for suspected coronary disease the prevalence was 2.2%.
The terms cleft and crypt have been used interchangeably to describe such recesses containing luminal blood into the otherwise normal compact myocardium, with a systolic narrowing or obliteration. In literature, small muscular diverticula have been sometimes (be) termed “crypts,” as it may not be easy to detect the exact percentage of myocardial fibers surrounding the recess.
Although crypts may be significant findings in a familial context with a high pretest probability of HCM, their clinical significance is doubtful when isolated findings in asymptomatic patients, probably representing, in these cases, only incidental variants of local myocardial structure.,,,
According to Erol et al., we believe that all these fissure-like lesions, sometimes called recesses, crypts or clefts are the same entity and maybe only the term crypts should be used to avoid confounding terminology but, again, an agreement is warranted.
Basso et al., in an Editorial comment to Petryka's paper, added the term “crevices,” but this definition of myocardial fissures does not seem to differ from clefts or crypts and has quite never been used according to the results of our PubMed research.
| Etiology|| |
CVD are developmental anomalies arising during the fourth embryonic week. The etiology of nonapical, isolated CVD and CVA has been attributed to a focal defect of the muscular ventricular wall due to intrinsic abnormality in embryogenesis. Congenital outpouchings may be acquired during foetal or neonatal period, due to a viral infection, coronary anomalies or vasculitis or arrhythmia-related vascular accidents, which may result in a localized weakness of the LV wall with gradual protrusion, due to the high intraventricular pressure.
Ventricular clefts, crypts, crevices, or fissures most frequently represent “a failure to resorb the trabeculated wall during embryological development phase in which the ventricular wall consists of a spongy meshwork of fibers organized into trabeculae with deep recesses communicating with the LV cavity and enabling a direct blood supply to the myocardium.” Spontaneous closure of a ventricular septal defect may resemble the diagnostic features of a septal cleft or diverticula. Kantarci et al. reviewed the records of 2.725 consecutive patients referred for cardiac CT scan and found septal outpouches in 18 cases. Judging from their aspect, the Authors supposed that these “pouches or sacs” may represent a consequence of spontaneous closure of muscular septal defects. Unfortunately, no further studies have investigated this topic.
Isolated ventricular noncompaction is another entity that needs to be differentiated. It is characterized by a prominent trabecular meshwork with a distinctly spongy appearance and deep inter-trabecular recesses commonly believed to be caused by an arrest in normal embryogenesis.,, The imaging features of ventricular diverticula and crypts can resemble ventricular noncompaction, and it has been proposed that at least the presence of more than three deep inter-trabecular recesses are necessary to diagnose a noncompaction. Moreover, Singh et al. hypothesized a continuum or a common developmental abnormality between diverticula and noncompaction. Some apical outpouchings may resemble a Takotsubo cardiomyopathy [Figure 1], and a differential diagnosis in the acute phase may be difficult due to the absence of delayed enhancement at CMR: The clinical setting and the reversibility of the dyskinesia are the main diagnostic features.
|Figure 1: Echocardiographic images of an apical congenital aneurysm. Echocardiographic apical four-chamber views. On the left, a diastolic still image displaying a congenital apical aneurysm with a large neck, surrounded by thin tissue, and with systolic expansion, on the right (yellow arrow). The patient was an asymptomatic 19-year-old woman with biphasic T waves on precordial leads V4–V6 and no delayed enhancement was present at cardiac magnetic resonance|
Click here to view
Malakan Rad et al. proposed a novel classification of LV outpouchings. According to their classification, if the normal elliptical shape of the left ventricular cavity is preserved, then the diagnosis should be DCLV., Apart from the complexity of this proposal, the inclusion of DCLV among CVOs is a questionable topic.
| Diagnosis|| |
Most patients remain asymptomatic and without complications throughout their lifetime and CVD, in most cases, are incidental echocardiographic findings. In Ohlow's series, 60% of the patients were asymptomatic. The most common symptoms were syncope and rhythm disturbances (43%), followed by chest pain (39%) and embolic events (2%). Symptomatic patients may have ventricular arrhythmias, such as sporadic premature ventricular contractions,,, but sudden cardiac death was also reported.
CVOs can be diagnosed in any age, with clinical finding ranging from prenatal rupture and death,, to completely asymptomatic presentation in late adulthood. The prognosis depends on the severity of underlying cardiac pathology and associated malformations. In adults, ventricular diverticula have usually a benign course.,,
Progressive enlargement and spontaneous rupture of a diverticulum have been described. Marijon demonstrated a mortality rate of 70% in a population of neonates and pediatric patients with associated other intra- and/or extra-cardiac malformations.
Ventricular arrhythmias in diverticula have been reported,, and syncope may be the first symptom. Ventricular tachycardia is often monomorphic with right bundle branch block morphology, often inducible during electrophysiological study and a cardioverter defibrillator may be required. Fellows et al. reported two patients with aborted sudden cardiac death and one patient who presented with syncope and documented nonsustained ventricular tachycardia. Shen et al. described a male patient with a sustained ventricular tachycardia caused by a postero-basal left ventricular diverticulum. Systemic embolism is a very rare complication, because of blood stasis in its cavity [Figure 2].
|Figure 2: Three-dimensional CT scan reconstruction of an apical diverticulum. (a and c): CT scan axial and oblique diastolic image (b and d): CT scan axial and oblique systolic image showing quite complete obliteration of the diverticulum. (e): echocardiographic appearance of the apical outpoching. (f): 3D CT-scan allows a clear view of the cavity with a tortuous diverticulum (yellow arrow). The patient was asymptomatic until at 56-year-old, he had cryptogenic” ischemic stroke (same case of Figures 3 and 6). LV = Left ventricle, RC = Right coronary artery, RV = Right ventricle, CT = Computed tomography, 3D = Three-dimensional|
Click here to view
Electrocardiogram (ECG) may be normal, but in about half of the cases, it shows incomplete bundle branch blocks or nonspecific ST-T changes and sometimes LV hypertrophy pattern. The largest CVD series has been described by Ohlow: about 56.8% of patients have ECG abnormalities and some may rise the suspicion of ventricular diverticula: (1) repolarization pattern with inverted T wave >2 mm in ≥2 contiguous leads; (2) repolarization patterns with either flat, minimally inverted, or >15 mm tall T waves in >2 leads; (3) Q waves 2–3 mm in depth and present in >2 leads; (4) abnormal R progression in the anterior precordial leads; (5) atrial fibrillation; (6) right bundle branch block; (7) early repolarization pattern; and (8) increased PR interval duration. More frequently, ECG abnormalities are found in the apical diverticula, sometimes suggesting left ventricular hypertrophy, a challenging pattern particularly in athletes, [Figure 3]. These ECG findings are not specific but may be symptomatic of a cardiac diverticulum or aneurysm in the absence of other possible ethiologies.
|Figure 3: Electrocardiogram of an athlete with an apical left ventricular diverticulum and signs of left ventricle hypertrophy. Repolarization abnormalities and a pattern of left ventricular hypertrophy are present in half of the cases of ventricular diverticula. In this case, a large apical diverticulum was present (same case of Figure 2). T wave inversion in the inferior and lateral precordial leads are present without elevation of the J-point (yellow arrows), and an incomplete right bundle branch block. These abnormalities should not be confused with early repolarization pattern and with an hypertrophic cardiomyopathy|
Click here to view
Most CVOs are incidentally found during an echocardiographic examination. Echocardiography may allow accurate detection of diverticula and assessment of their location, morphology, contractility, presence of thrombosis, and associated congenital cardiac disorders. A CVD appears as a tongue-like or finger-shaped outpouching of the ventricular wall. A narrow neck and a finger-like contractile pouch are typical characteristics of the congenital muscular ventricular diverticulum, [Figure 4]. Small muscular diverticula and clefts cavity may completely disappear during systole; therefore, only telediastolic images should be used to define diagnosis and measurement, [Figure 5]. Echocardiographic visualization can be challenging, particularly if the CVO location does not coincide with standard acquisition planes. Contrast-enhanced harmonic imaging is a very helpful tool as the contrast entering the recess clearly delineate its contour.
|Figure 4: Echocardiographic displays of a left ventricular diverticulum in the inferolateral wall. On the left an apical two chamber and on the right a parasternal short axis view. The diverticulum has a narrow neck and appears as a contractile finger-like pouch in the entire left ventricular wall. The inferior, lateral wall and the apex are the preferred localization. LA = Left atrium, LV = Left ventricle, PML = Posterior mitral leaflet, RV = Right ventricle|
Click here to view
|Figure 5: Echocardiographic characteristics of a left ventricular cleft. During diastole (left) a narrow neck deep pouch is seen in the inferior wall, but it almost disappears during systole (right) because of the contraction of the surrounding muscular fibers (yellow arrow). The indentation exceeds half the myocardial thickness without reaching the epicardium. LV = Left ventricle|
Click here to view
Angiographic ventriculography offers another diagnostic option, but due to its invasive nature is less useful than other imaging modalities and it may be used to confirm diagnosis in patients with chest pain undergoing coronary angiography. Minor coronary arteries anomalies may be associated to CVD, and are described in up to 58.1% of the cases with a 9-fold risk. This high percentage is somewhat surprising, and the Authors themselves admit a possible selection bias. The most frequent coronary anomaly was a splitting of the origin followed by an ectopic origin but no major anomalies, with potential lethal consequences, have been found.
The high spatial and temporal resolution offered by ECG-gated cardiac CT provides a unique opportunity to evaluate the ventricular walls., The detection of asymptomatic clefts appearing as V-shaped perpendicular recesses penetrating more than 50% of the myocardial thickness, usually with normal adjacent muscle, seems to be particularly frequent, [Figure 2]. Whether a CT scan may be always advisable in patients with ventricular outpouchings is a matter of debate. Due to high prevalence of minor coronary anomalies and in order to exclude secondary causes of ventricular aneurysms, a coronaric CT scan should be performed. Moreover, a CT scan should be performed when the echocardiographic examination is not conclusive, and CMR not available or contraindicated.
CMR is the ideal noninvasive and nonionizing diagnostic tool. Black blood and steady-state free precession sequences respectively provide accurate definition, measurement, tissue characterization, and contractility assessment [Figure 6], [Figure 7], [Figure 8]. The depth of the outpouching in diastolic frames may be exactly evaluated in order to differentiate whether it is <50% of the wall thickness. The detection of delayed enhancement with T1-weighted sequences, allows diagnosis of fibrous or necrotic tissue and thus the differential diagnosis between diverticula and aneurysms.,,, A pseudoaneurysm is composed only of pericardium and does not show delayed enhancement, while the presence of enhancement around outpouching indicates a peri-aneurysmal necrotic wall. We, therefore, recommend performing a CMR as it is the gold standard imaging technique in the suspect of a CVO.
|Figure 6: Cardiac Magnetic Resonance images of an apical ventricular diverticulum (same case of Figure 2) Panel A: T1-weighted four-chamber view Panel B: Perfusion imaging still frame four-chamber view showing the contrast media filling the diverticulum cavity Panel C: The gadolinium delayed image does not show late enhancement|
Click here to view
|Figure 7: Cardiac magnetic resonance images of a left ventricular diverticulum localized in the inferior wall. On the left a still frame of a cine-magnetic resonance imaging sequence and on the right the late gadolinium enhancement sequence. The diverticulum is deep and extends beyond the myocardial wall, has a narrow neck and is surrounded by vital myocardial tissue, without scar as demonstrated by the absence of late gadolinium enhancement. This allows the differential diagnosis with ischemic aneurysm or pseudo-aneurysm (yellow arrow). LV = Left ventricle. The patient was an asymptomatic 15-year-old male|
Click here to view
|Figure 8: Cardiac magnetic resonance images: left ventricular cleft of the basal inferior wall in a 69 yo woman affected by Hypertrophic Cardiomyopathy. Panel A: T1-weighted two-chamber views: the cleft extends beyond the half of the wall Panel B: Cine image still diastolic frame of the same two-chamber view Panel C: Cine image still systolic frame showing the complete disappearance of the outpouching|
Click here to view
| Proposed Classification|| |
In addition to the anamnestic data of a previous myocardial injury, a multi-imaging approach can differentiate diverticula from aneurysms: [Table 3] and [Figure 9] shows our proposed classification: diverticula have a narrow neck and contractility, the ejection fraction is usually normal, there is a systolic flow pattern from the outpouching to the ventricle, the size is often smaller and diastolic shape more circular. In contrast, a left ventricular aneurysm has a wide neck with akinetic walls or paradoxical expansion during systole, no myocardial muscle layer and a layer of fibrous tissue which may be calcified as detectable by the late gadolinium enhancement sequences [Figure 6]. The main diagnostic feature to differentiate diverticula from crypts is that CVD extend outside the epicardial border while crypts remain confined inside the myocardial margin, [Table 2].
|Table 3: Differential diagnosis between congenital ventricular outpouchings|
Click here to view
|Figure 9: Recess has narrow neck and does not reach half of myocardial wall while crypt (or cleft) goes beyond half of the wall. In systole they obliterate. Diverticulum has narrow neck, coated by thinned vital myocardium and goes beyond the myocardial wall; during systole, it contracts but does not completely disappear. The Congenital Aneurysm has broad neck, coated by fibrous tissue with systolic dyskinesia. The pseudoaneurysm is due to myocardial ischemia with wall rupture and blood in pericardium. The Double-Chambered left ventricle is a rare congenital abnormality in which left ventricle has an anomalous septum or band|
Click here to view
CVD and CVA must be also differentiated from pseudoaneurysms, which are acquired complication of myocardial infarction, cardiac surgery, trauma, or infection. Pseudoaneurysms, also termed false aneurysms, are the consequences of a rupture of the ventricular wall contained by overlying fibrosis and thrombosis of the adherent pericardium.
| Conclusion|| |
Our review confirmed the need of an expert consensus to avoid confounding terminologies used to define CVOs. A simple classification may be proposed: Diverticula extend beyond the myocardial wall, and fibrous type may be termed congenital aneurysm, acquired forms should be kept distinct; the term clefts, or crypts, might be used to describe small muscular recesses extending for more than 50% of the ventricular wall but not beyond its margin. CMR seems to be the imaging modality of choice due to the possibility of evaluating the presence of fibrosis and to exclude dubious forms of HCM or ventricular noncompaction.
A multicenter prospective registry, imaged by CMR and/or CT scans, and a long-term follow-up would be needed to investigate clinical implications.,,,
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Erol C, Koplay M, Olcay A, Kivrak AS, Ozbek S, Seker M, et al.
Congenital left ventricular wall abnormalities in adults detected by gated cardiac multidetector computed tomography: Clefts, aneurysms, diverticula and terminology problems. Eur J Radiol 2012;81:3276-81.
Malakan Rad E, Awad S, Hijazi ZM. Congenital left ventricular outpouchings: A systematic review of 839 cases and introduction of a novel classification after two centuries. Congenit Heart Dis 2014;9:498-511.
Coene Bales A, Sandelski J, Sareli P, Lang RM. Left ventricular diverticula and aneurysms: Congenital and acquired lesions. Echocardiography 1998;15:77-88.
Chen HW, Chen ML, Yang B, Ju WZ, Zhang FX, Hou XF, et al.
Clinical characteristics of congenital ventricular aneurysm and diverticula in inland China. Zhonghua Xin Xue Guan Bing Za Zhi 2011;39:865-8.
Moher D, Liberati A, Tetzlaff J, Altman DG; PRISMA Group. Preferred reporting items for systematic reviews and meta-analyses: The PRISMA statement. J Clin Epidemiol 2009;62:1006-12.
Ohlow MA. Congenital left ventricular aneurysms and diverticula: Definition, pathophysiology, clinical relevance and treatment. Cardiology 2006;106:63-72.
Peacock TB, editor. On Malformations of the Human Heart, etc.: With Original Cases and Illustrations. London: John Churchill and Sons; 1866.
Tullu MS, Vaideeswar P, Deshmukh CT. Congenital left ventricular diverticula. Int J Cardiol 2000;73:293-5.
Makkuni P, Kotler MN, Figueredo VM. Diverticular and aneurysmal structures of the left ventricle in adults: Report of a case within the context of a literature review. Tex Heart Inst J 2010;37:699-705.
Nam KH, Kwon JY, Son GH, Cho NH, Park YW, Kim YH, et al.
Prenatally diagnosed left ventricular diverticulum with thoracoabdominal wall defect: A case and review of the literature. J Perinatol 2010;30:760-2.
Srichai MB, Hecht EM, Kim DC, Jacobs JE. Ventricular diverticula on cardiac CT: More common than previously thought. AJR Am J Roentgenol 2007;189:204-8.
Mady C. Left ventricular diverticulum: Analysis of two operated cases and review of the literature. Angiology 1982;33:280-6.
McMahon CJ, Moniotte S, Powell AJ, del Nido PJ, Geva T. Usefulness of magnetic resonance imaging evaluation of congenital left ventricular aneurysms. Am J Cardiol 2007;100:310-5.
Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet 1958;107:602-14.
Hubacek J, Brydie A, Jackson S. Multiple congenital left ventricular diverticula and aneurysm. J Am Coll Cardiol 2009;53:1087.
Archbold RA, Robinson NM, Mills PG. Long-term follow-up of a true contractile left ventricular diverticulum. Am J Cardiol 1999;83:810-3, A11.
Takahashi M, Nishikimi T, Tamano K, Hara S, Kobayashi T, Honda T, et al.
Multiple left ventricular diverticula detected by second harmonic imaging: A case report. Circ J 2003;67:972-4.
Shauq A, Agarwal V, Crawley C. Congenital left ventricular diverticulum. Heart Lung Circ 2006;15:272-4.
Skapinker S. Diverticulum of the left ventricle of the heart; review of the literature and report of a successful removal of the diverticulum. AMA Arch Surg 1951;63:629-34.
Aquaro GD, Strata E, Di Bella G, Todiere G, Pugliese N, Del Franco A, et al.
Prognostic role of isolated left ventricular diverticuli detected by cardiovascular magnetic resonance. J Cardiovasc Med (Hagerstown) 2015;16:562-7.
Nakazono T, Jeudy J, White CS. Left and right ventricular diverticula: Incidence and imaging findings on 256-slice multidetector computed tomography. J Thorac Imaging 2012;27:179-83.
Du Toit HJ, Von Oppell UO, Hewitson J, Lawrenson J, Davies J. Left ventricular sub-valvar mitral aneurysms. Interact Cardiovasc Thorac Surg 2003;2:547-51.
Marijon E, Ou P, Fermont L, Concordet S, Le Bidois J, Sidi D, et al.
Diagnosis and outcome in congenital ventricular diverticulum and aneurysm. J Thorac Cardiovasc Surg 2006;131:433-7.
Petryka J, Baksi AJ, Prasad SK, Pennell DJ, Kilner PJ. Prevalence of inferobasal myocardial crypts among patients referred for cardiovascular magnetic resonance. Circ Cardiovasc Imaging 2014;7:259-64.
Kuribayashi T, Roberts WC. Myocardial disarray at junction of ventricular septum and left and right ventricular free walls in hypertrophic cardiomyopathy. Am J Cardiol 1992;70:1333-40.
Johansson B, Maceira AM, Babu-Narayan SV, Moon JC, Pennell DJ, Kilner PJ, et al.
Clefts can be seen in the basal inferior wall of the left ventricle and the interventricular septum in healthy volunteers as well as patients by cardiovascular magnetic resonance. J Am Coll Cardiol 2007;50:1294-5.
Germans T, Wilde AA, Dijkmans PA, Chai W, Kamp O, Pinto YM, et al.
Structural abnormalities of the inferoseptal left ventricular wall detected by cardiac magnetic resonance imaging in carriers of hypertrophic cardiomyopathy mutations. J Am Coll Cardiol 2006;48:2518-23.
Ozturk E, Saglam M, Sivrioglu AK, Kara K. Left ventricular clefts and diverticula. Eur J Radiol 2013;82:e628.
Moon JC, McKenna WJ. Myocardial crypts: A prephenotypic marker of hypertrophic cardiomyopathy? Circ Cardiovasc Imaging 2012;5:431-2.
Moon JC, Reed E, Sheppard MN, Elkington AG, Ho SY, Burke M, et al.
The histologic basis of late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy. J Am Coll Cardiol 2004;43:2260-4.
Maron MS, Rowin EJ, Lin D, Appelbaum E, Chan RH, Gibson CM, et al.
Prevalence and clinical profile of myocardial crypts in hypertrophic cardiomyopathy. Circ Cardiovasc Imaging 2012;5:441-7.
Basso C, Marra MP, Thiene G. Myocardial clefts, crypts, or crevices: Once again, you see only what you look for. Circ Cardiovasc Imaging 2014;7:217-9.
Papagiannis J, Van Praagh R, Schwint O, D'Orsogna L, Qureshi F, Reynolds J, et al.
Congenital left ventricular aneurysm: Clinical, imaging, pathologic, and surgical findings in seven new cases. Am Heart J 2001;141:491-9.
Brachlow A, Sable C, Smith S, Slack M, Martin G. Fetal diagnosis and postnatal follow-up of an asymptomatic congenital left ventricular diverticulum. Pediatr Cardiol 2002;23:658-60.
Paronetto F, Strauss L. Aneurysm of the left ventricle due to congenital muscle defect in an infant. report of a case with discussion of pathogenesis of associated endocardial fibroelastosis. Am J Cardiol 1963;12:721-9.
Kantarci M, Olgun H, Duran C. Is it ventricular diverticulum or closed muscular ventricular septal defect? AJR Am J Roentgenol 2008;190:W374.
Kantarci M, Duran C, Bozkurt M, Guven F, Ceviz N, Sagsoz M, et al.
Cardiac multidetector computed tomography (MDCT) of spontaneously closed ventricular septal defect. Radiol Med 2009;114:370-5.
Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Arnett D, et al.
Contemporary definitions and classification of the cardiomyopathies: An American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation 2006;113:1807-16.
Hamamichi Y, Ichida F, Hashimoto I, Uese KH, Miyawaki T, Tsukano S, et al.
Isolated noncompaction of the ventricular myocardium: Ultrafast computed tomography and magnetic resonance imaging. Int J Cardiovasc Imaging 2001;17:305-14.
Singh Y, Singh B, Bhairappa S, Subramani KS, Prasad NM, C Nanjappa M, et al.
Arare association of left ventricular diverticulum and noncompaction: Continuum of common developmental abnormality? Echocardiography 2013;30:E171-4.
Wang M, Li YJ, Yang R, Zhang H. A congenital isolated left ventricular apical diverticulum simulating a tako-tsubo. Chin Med J (Engl) 2011;124:315-7.
Mordi I, Carrick D, Tzemos N. Diagnosis of double-chambered left ventricle using advanced cardiovascular imaging. Echocardiography 2013;30:E206-8.
Maloy WC, Arrants JE, Sowell BF, Hendrix GH. Left ventricular aneurysm of uncertain etiology with recurrent ventricular arrhythmias. N
Engl J Med 1971;285:662-3.
Fellows CL, Bardy GH, Ivey TD, Werner JA, Draheim JJ, Greene HL, et al.
Ventricular dysrhythmias associated with congenital left ventricular aneurysms. Am J Cardiol 1986;57:997-9.
Shen EN, Fukuyama O, Herre JM, Yee E, Scheinman MM. Ventricular tachycardia with congenital ventricular diverticulum. Chest 1991;100:283-5.
Uchida T, Uemura H, Yagihara T, Kawahira Y, Yoshikawa Y, Kitamura S, et al.
Congenital diverticulum of the left ventricle. Jpn J Thorac Cardiovasc Surg 2001;49:244-6.
Li Q, Qu H, Wang H, Wang D, Li P, Liu T, et al.
Ventricular diverticulum: A review of the literature. J Card Surg 2013;28:133-8.
Walton-Shirley M, Smith SM, Talley JD. Left ventricular diverticulum: Case report and review of the literature. Cathet Cardiovasc Diagn 1992;26:31-3.
Gorgels AP. No value of the ECG in congenital left ventricular aneurysms and diverticula? Europace 2009;11:1577-8.
Haegeli LM, Ercin E, Steffel J, Wolber T, Tanner FC, Jenni R, et al.
Incidence and prognosis of ventricular arrhythmias in patients with congenital left ventricular aneurysms or diverticula. Am J Med 2015;128:653.e1-6.
Bell WE, Ehmke DA. Diverticulum of the left ventricle in a child with fatal cerebral embolization. South Med J 1971;64:537-40.
Kumbasar B, Wu KC, Kamel IR, Lima JA, Bluemke DA. Left ventricular true aneurysm: Diagnosis of myocardial viability shown on MR imaging. AJR Am J Roentgenol 2002;179:472-4.
Kim RJ, Fieno DS, Parrish TB, Harris K, Chen EL, Simonetti O, et al.
Relationship of MRI delayed contrast enhancement to irreversible injury, infarct age, and contractile function. Circulation 1999;100:1992-2002.
Cianciulli TF, Del Carmen Gonzalez Colaso P, Saccheri MC, Lax JA, Redruello HJ, Guerra JE, et al.
Left ventricular diverticulum, a rare echocardiographic finding: Two adult patients and review of the literature. Cardiol J 2009;16:76-81.
Ropers D, Achenbach S, Pfeiffer S. Left ventricular pseudoaneurysm following myocardial infarction. Heart 2004;90:555.
van Brabandt H, Piessens J, Stalpaert J, de Geest H. Pseudoaneurysm of the left ventricle following cardiac surgery. Report of 3 cases and review of the literature. Thorac Cardiovasc Surg 1985;33:118-24.
Solaković E, Pasić M. Pseudoaneurysm in the posterior wall of the left ventricle with perforation of the left ventricle due to a firearm injury. Med Arh 2004;58:125-6.
Yamabi H, Imanaka K, Tanabe H, Abe K, Shimamura Y, Asano H, et al.
Pseudoaneurysm of the left ventricle following suppurative pericarditis and sepsis due to Staphylococcus aureus
: A case report. J Cardiol 2004;44:119-22.
Ichikawa K, Makino K, Futagami Y, Fujioka H, Ito M, Hamada M, et al
. Isolated congenital left ventricular diverticulum in an adult. A case report. Angiology 1994;45:743-7.
Choi CH, Elahi MM, Konda S. Iatrogenic retained foreign body in the right atrium. Lessons to Learn. International Journal of Surgery Case Reports 2013;4:985-7.
Mayer K, Candinas R, Radounislis C, et al
. Congenital left ventricular aneurysms and diverticula: Clinical findings, diagnosis and course [In German]. Schweiz Med Wochenschr 1999;129:1249-56.
Child N, Muhr T, Sammut E, Dabir D, Arroyo Ucar E, Bueser T. Prevalence of myocardial crypts in a large retrospective cohort study by cardiovascular magnetic resonance. Journal of Cardiovascular Magnetic Resonance 2014;16:66.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]
[Table 1], [Table 2], [Table 3]
|This article has been cited by|
||Echocardiographic features of left ventricular recess, cleft, diverticulum, and aneurysm: A systematic review
| ||Kjell Høyland, Abukar Mohamed Ali, Johnny Vegsundvåg, John B. Chambers, Sahrai Saeed |
| ||Journal of Clinical Ultrasound. 2022; |
|[Pubmed] | [DOI]|
||How to look at adult congenital left ventricular outpouchings: a step-by-step approach using cardiac magnetic resonance
| ||Francesco Cardaioli, Giulia Brunetti, Alberto Cipriani, Raffaella Motta, Giuseppe Tarantini, Giovanni Di Salvo, Gaetano Thiene, Domenico Corrado, Cristina Basso, Sabino Iliceto, Manuel De Lazzari, Martina Perazzolo Marra |
| ||European Heart Journal - Cardiovascular Imaging. 2022; |
|[Pubmed] | [DOI]|
||Dilated left ventricle with multiple outpouchings — a severe congenital ventricular diverticulum or left-dominant arrhythmogenic cardiomyopathy: A case report
| ||Xin Zhang, Run-Yu Ye, Xiao-Ping Chen |
| ||World Journal of Clinical Cases. 2022; 10(18): 6289 |
|[Pubmed] | [DOI]|
||Arrhythmogenic Right Ventricular Cardiomyopathy and Differential Diagnosis with Diseases Mimicking Its Phenotypes
| ||Nadine Molitor, Firat Duru |
| ||Journal of Clinical Medicine. 2022; 11(5): 1230 |
|[Pubmed] | [DOI]|
||Differential diagnosis of arrhythmogenic cardiomyopathy: phenocopies versus disease variants
| ||Alberto CIPRIANI,Martina PERAZZOLO MARRA,Riccardo BARIANI,Giulia MATTESI,Riccardo VIO,Natascia BETTELLA,Manuel DE LAZZARI,Raffaella MOTTA,Barbara BAUCE,Alessandro ZORZI,Domenico CORRADO |
| ||Minerva Medica. 2021; 112(2) |
|[Pubmed] | [DOI]|
||The Role of Cardiac Magnetic Resonance in Evaluation of Idiopathic Ventricular Arrhythmia in Children
| ||Halszka Kaminska,Lukasz A. Malek,Marzena Barczuk-Falecka,Marta Bartoszek,Ewa Strzalkowska-Kominiak,Mikolaj Marszalek,Ewa Brzezik,Michal Brzewski,Bozena Werner |
| ||Journal of Clinical Medicine. 2021; 10(7): 1335 |
|[Pubmed] | [DOI]|
||Description and Clinical Implications of Myocardial Clefts Using Echocardiography
| ||Ghaith Alhatemi,Aditya Sood,Haider Aldiwani,Rafal Alhatemi,Abdelrahman Ahmed,Mohamed Shokr,Mohamed Zghouzi,M. Chadi Alraies,Shaun Cardozo |
| ||Cureus. 2021; |
|[Pubmed] | [DOI]|
||Cardiac Outpouchings: Definitions, Differential Diagnosis, and Therapeutic Approach
| ||Riccardo Scagliola,Gian Marco Rosa,Sara Seitun,Luigi Sciarra |
| ||Cardiology Research and Practice. 2021; 2021: 1 |
|[Pubmed] | [DOI]|
||Right atrial appendage aneurysm in a fetus—Does precise prenatal diagnosis matter?
| ||Olga Ivanitskaya,Yulia Sologub,Yulia Tsayuk,Alexey Zagray,Aleksei Kim |
| ||Ultrasound. 2020; 28(4): 255 |
|[Pubmed] | [DOI]|
||Incidental detection of myocardial clefts in a patient with acute inferior ST-segment elevation myocardial infarction: a very unusual and potentially ominous association—a case-report
| ||Giovanni Garau,Yoann Bataille,Eric Larose,Etienne Hoffer,Linh Ngo,Dias Andre,Miglioranza Marcelo Haertel,Arcari Luca,Vervaat Fabienne |
| ||European Heart Journal - Case Reports. 2020; |
|[Pubmed] | [DOI]|
||Coil occlusion of a congenital left ventricular aneurysm in a newborn
| ||Michael Hofbeck,Ludger Sieverding,Gerd Grözinger,Karl O. Kagan |
| ||Catheterization and Cardiovascular Interventions. 2020; |
|[Pubmed] | [DOI]|
||Accessory left ventricular chamber in a cat: multimodality imaging description by cardiac magnetic resonance imaging and echocardiography
| ||J. Bouvard,M. Longo,T. Schwarz,Y. Martinez Pereira |
| ||Journal of Veterinary Cardiology. 2020; 28: 55 |
|[Pubmed] | [DOI]|
||Diagnóstico ante una deformación sacular del ventrículo izquierdo en niños. No todo es lo que parece
| ||Juan Manuel Carretero Bellon,Alex Pérez Casares,Laia Brunet García,Fredy Hermogenes Prada,Joan Sánchez de Toledo |
| ||Revista de ecocardiografía práctica y otras técnicas de imagen cardíaca. 2020; 3(3): 6 |
|[Pubmed] | [DOI]|
||Three-dimensional transthoracic echocardiographic imaging of an unusual congenital left ventricular diverticulum
| ||Keisuke Kawai,Hiroshi Watanabe,Minoru Hasokawa,Masakazu Nanahoshi,Hiroshi Yamabe,Seiichi Kobayashi |
| ||Journal of Echocardiography. 2019; |
|[Pubmed] | [DOI]|
||Phenotypes of hypertrophic cardiomyopathy. An illustrative review of MRI findings
| ||Rafaela Soler,Cristina Méndez,Esther Rodríguez,Roberto Barriales,Juan Pablo Ochoa,Lorenzo Monserrat |
| ||Insights into Imaging. 2018; |
|[Pubmed] | [DOI]|