Congenital Ventricular Diverticulum Research Articles

Prenatal features and postnatal follow-up of congenital ventricular outpouching: A retrospective study of two centers in China.

Congenital ventricular outpouching (CVO) is a rare cardiac malformation that can manifest as congenital ventricular aneurysm (CVA) and/or congenital ventricular diverticula (CVD). In this study, we describe the prenatal features and postnatal follow-up of 27 cases of CVO. The clinical data of 27 patients with CVO who attended Sir Run Run Shaw Hospital Affiliated to the Medical College of Zhejiang University (Zhejiang Province, China) and Taizhou Hospital of Zhejiang Province Affiliated to Wenzhou Medical University (Zhejiang Province, China) from April 2013 to October 2022 were retrospectively analyzed. The patients were also followed up by telephone. The prenatal characteristics and postnatal outcomes of the patients with CVO were evaluated. CVO was detected in 26 cases prenatally, 14 (51.85%) were diagnosed with CVA, nine (33.33%) were diagnosed with CVD, three (11.11%) were equivocal for CVA/CVD, and one (3.70%) was detected with CVA postnatally. Six patients underwent follow-up fetal echocardiography approximately 4weeks after the initial echocardiography examination, and a significant difference in CVO size was observed between the two examinations (P=.02). Eight patients (29.63%) demonstrated cardiovascular dysfunction, and the median CVO size in fetuses with and without cardiovascular dysfunction was 205 (range: 169-396) mm2 and 124 (range: 92-154.5) mm2 , respectively (P=.01). Correlation was found between CVO size and fetal cardiac dysfunction (p=.000, r=.778). Eight patients (29.63%) had cardiac/extracardiac defects. Thirteen patients were live born, 12 were terminated pregnancies, and two were lost to follow-up. The postpartum size of the CVOs remained stable in six patients, decreased in two patients, dissolved in three patients, and were surgically removed in two patients. With the exception of one patient with CVA complicated with complex congenital cardiac malformation who underwent surgical treatment after birth and who had postoperative left ventricular dysfunction (Case 1), the prognosis of all of the patients was good. CVO is often associated with cardiac malformations. The size of prenatal CVOs can increase with gestational development, and cardiovascular dysfunction is significantly related to CVO size. The postpartum prognosis of patients with CVO is good. Echocardiography plays a key role in the diagnosis of congenital ventricular outpouching. Prenatal counseling should be cautious regarding the diagnosis and the prognosis although our cases had a favorable prognosis.

Characteristics and outcomes of fetal ventricular aneurysm and diverticulum: combining the use of a new technique, fetal HQ.

Congenital ventricular aneurysms or diverticulum (VA/VD) are rare cardiac anomalies with lack prenatal evaluation data. The present study aimed to provide the prenatal characteristics and outcomes from a tertiary center and the use of new techniques to evaluate the shape and contractility of these fetuses. Ten fetuses were diagnosed with VA or VD, and 30 control fetuses were enrolled. Fetal echocardiography was performed to make the diagnosis. The prenatal echo characteristics and follow-up data were carefully reviewed. The shape and contractility measurements of the four-chamber view (4CV) and both ventricles were measured and computed using fetal fetal heart quantification (HQ). A total of 10 fetuses were enrolled, including 4 cases of left ventricular diverticulum, 5 cases of left ventricular aneurysm, and 1 case of right ventricular aneurysm (RVA). Four cases chose to terminate the pregnancy. The RVA was associated with a perimembranous ventricular septal defect. Two cases had fetal arrhythmia, and one case had pericardial effusion. After birth, one case underwent surgical resection at five years old. The 4CV global sphericity index (SI) of free-wall located ventricular outpouching (VO) was significantly lower than the apical ones and the control group (p < 0.01). Four of five apical left VOs had significant higher (>95th centile) SI in base segments, and three of four left VOs in the free-wall had significant lower (< 5th centile) SI in the majority of 24 segments. Compared to the control group, the left ventricle (LV) global longitudinal strain, ejection fraction, and fractional area change were significantly decreased (p < 0.01), while the LV cardiac output of the cases was in the normal range. The transverse fraction shortening of the affected segments of ventricles was significantly lower than the other ventricle segments (p < 0.01). Fetal HQ is a promising technique to evaluate the shape and contractility of congenital ventricular aneurysm and diverticulum.

Prenatal diagnosis of right-sided congenital ventricular diver-ticulum (CVD) assisted by spatiotemporal image correlation (STIC) acquisition and the speckle-tracking technique to as-sess fetal cardiac function: A case report

Prenatal diagnosis of right-sided congenital ventricular diver-ticulum (CVD) assisted by spatiotemporal image correlation (STIC) acquisition and the speckle-tracking technique to as-sess fetal cardiac function: A case report

Prenatal diagnosis of right-sided congenital ventricular diver-ticulum (CVD) assisted by spatiotemporal image correlation (STIC) acquisition and the speckle-tracking technique to as-sess fetal cardiac function: A case report

Prenatal diagnosis of right-sided congenital ventricular diver-ticulum (CVD) assisted by spatiotemporal image correlation (STIC) acquisition and the speckle-tracking technique to as-sess fetal cardiac function: A case report

Congenital biventricular cardiac diverticula as a part of class III Cantrell's syndrome: case report.

Congenital cardiac ventricular diverticulum is an extremely rare condition that usually occurs as a part of Pentalogy of Cantrell and frequently associated with sternal, pericardial, diaphragmatic, and thoracoabdominal wall defects. The prognosis of the patient depends on the complexity of abnormalities. Herein, we report biventricular diverticula as a part of incomplete Cantrell's syndrome in a 1-day-old newborn.

Prenatal diagnosis of congenital ventricular aneurysm and diverticulum: Prenatal features and perinatal management.

We wanted to describe prenatal features and post-natal outcomes of fetal cardiac out pouching. We retrospectively report 6 cases of VD and aneurysm prenatally managed in our fetal medicine unit between 2010 and 2020. All cases were evaluated from the first or second trimester of pregnancy until postnatal follow-up (3months to 3years). All six cases underwent a monthly ultrasound follow-up with spontaneous regression of pericardial effusion, and normal hemodynamics at birth No pericardial puncture was done and postnatal outcome was favorable in all cases. Based on our experience and on cases previously published, prenatal counseling should be prudent regarding the final diagnosis. Referral and monthly prenatal ultrasound follow-up, birth in a tertiary center after multidisciplinary evaluation and cardiological evaluation at birth still seem mandatory.

Autopsy and postnatal follow-up of prenatally diagnosed ventricular outpouchings.

Prenatal ventricular outpouchings (VOs), which include congenital ventricular aneurysms (CAs) and congenital ventricular diverticula (CD), are very rare. We describe the features and outcomes of prenatal VOs diagnosed over a 4-year period. Retrospective cohort study of cases of prenatal diagnoses of CAs and CD at our center between June 2014 and January 2018. The prenatal and postnatal echocardiogram data were reviewed, and telephone follow-up was conducted of liveborn cases. A total of 25 VOs were identified. Two were lost to follow-up, 15 chose termination of pregnancy, and eight resulted in livebirths. Only two cases underwent autopsy: Histopathology showed that the CA wall was substituted by collagen fibers. At follow-up, none of the eight liveborn babies experienced adverse events, and three VOs near the tricuspid annulus almost disappeared, though one was extremely large. In our center, all liveborn babies with VO had good prognoses. We hypothesize that VOs located near the right ventricular annulus may be caused by prenatally unbalanced pressure, given their decrease in size after birth when the right heart pressure declines.

Congenital right ventricular diverticulum: An atypical presentation.

Congenital ventricular diverticulum is a rare congenital malformation of the heart. It remains mostly asymptomatic unless associated with other congenital cardiac defects. We present a case of a 4-month-old child who had dextrocardia, large inlet ventricular septal defect, amounting to single ventricle, and severe pulmonary artery hypertension. There was a right ventricular diverticulum which passed through the foramina of Morgagni to give rise to a pulsating lump in the epigastrium. Right ventricular diverticulum presenting with a pulsating mass in the abdomen is a rare entity. The confirmation of diverticulum was made with histopathology.

P1333 The importance of multimodality imaging in the characterization of a congenital myocardial diverticulum

Abstract Congenital ventricular diverticulum is a rare cardiac malformation, usually detected on echocardiographic examinations in asymptomatic patients. However, they can also cause embolisms, arrhythmias and sudden death due to ventricular rupture. Multimodality imaging can help in the characterization of its morphology and in the differential diagnosis. We report a case of an 83-year-old woman, pacemaker carrier due to complete atrioventricular block, who consulted for paroxysmal episodes of palpitations, being diagnosed of atrial fibrillation. A transthoracic echocardiogram was performed, observing an appendix at basal septal level with contractility of its wall and with doppler-colour inside, suggestive of myocardial diverticulum. Echocardiographic contrast was injected for better delimitation and characterization of the cavity. MRI was after performed for better characterization of this structure, confirming the presence of an accessory chamber with muscular wall and normal contractility at the level of the basal interventricular septum, without pathological enhancements. We reviewed an angioCT performed for other reasons some years ago, confirming the presence of the same structure, which went unnoticed in that study. The patient followed controls and remained asymptomatic, without evolutionary echocardiographic changes, so that a conservative attitude was maintained. Congenital ventricular diverticulum is formed by an appendix of the entire left ventricular (LV) wall. It can be differentiated into two types: muscular (more frequent, not prone to rupture and associated with other congenital malformations) or fibrous (frequently localized at the base of the heart or in subvalvular areas). Differential diagnosis may include true LV aneurysms (abnormal LV contour with systolic dyskinesia and involving thinned fibrous tissue) and LV pseudoaneurysms (acute contained rupture of the ventricle wall, often after myocardial infarction, also akinetic or dyskinetic). Congenital cardiac diverticulum is characterized by a rapid contrast filling and a narrow neck. MRI provide tissue characterization and is an important tool that helps in the differential diagnosis. In cardiac diverticulums, a thinned but contractile wall is present, without pathological delayed enhancements. In true aneurysms, the wall shows delayed enhancement (scar tissue). Pseudoaneurysms are only composed by pericardium, and the border of the aneurysms show enhancement indicating peri-aneurysmal infarcted area. Left ventricular catheterization can also be useful assessing the morphology and dynamics of the left ventricular chamber. In small, muscular and asymptomatic diverticulums, conservative treatment with follow-up is often recommended. When symptoms appear, medical or surgical treatment should be proposed. Abstract P1333 Figure. Congenital myocardial diverticulum

Multiple congenital ventricular diverticula causing heart failure

This paper reports a case of multiple congenital ventricular diverticula, a rare anomaly that is associated with heart failure.

Multimodality Imaging and Clinical Significance of Congenital Ventricular Outpouchings: Recesses, Diverticula, Aneurysms, Clefts, and Crypts.

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.

Congenital left ventricular aneurysms and diverticula: an entity in search of an identity.

Congenital left ventricular aneurysm or diverticulum are rare cardiac malformations described in 809 cases since the first description in 1816, being associated with other cardiac, vascular or thoraco-abdominal abnormalities in about 70%. It appears to be a developmental anomaly, starting in the 4th embryonic week. In an experimental study, targeted knockdown of cardiac troponin T in the chick was performed at day 3, after the heart tube has formed. Morpholino treatment of gene TNNT2 at this stage led to the development of left ventricular diverticula (LVD) in the primitive left ventricular wall. Diagnosis of left ventricular aneurysms (LVA)/LVD can be made after exclusion of coronary artery disease, local or systemic inflammation or traumatic causes as well as cardiomyopathies. Clinically, most of LVA and LVD are asymptomatic or may cause systemic embolization, congestive heart failure, valvular regurgitation, ventricular wall rupture, ventricular tachycardia or sudden cardiac death. Diagnosis is established by imaging studies (echocardiography, magnetic resonance imaging or left ventricular angiography) visualizing the structural changes and accompanying abnormalities. Mode of treatment has to be individually tailored and depends on clinical presentation, accompanying abnormalities and possible complications, options include surgical resection (especially in symptomatic patients), anticoagulation after systemic embolization, radiofrequency ablation or implantation of an implantable cardioverter defibrillator (ICD) in case of symptomatic ventricular tachycardias, and occasionally combined with class I- or III-antiarrhythmic drugs. Cardiac death occurs usually in childhood, is significantly more frequent in LVA patients and caused by congestive heart failure in most of the cases, whereas patients diagnosed with LVD died more frequently from rupture of the LVD.

Left ventricular diverticulum with ventricular premature complexes in fetal and postnatal period: A rare presentation

Congenital ventricular diverticulum is a rare congenital anomaly, which is defined as an outpouching structure that contains all the three layers of heart; that is, endocardium, myocardium, and pericardium and shows synchronous contractility. It remains clinically asymptomatic. The treatment of the left ventricular diverticulum (LVD) is undefined. We report a rare case of LVD presented as fetal bradycardia and further postnatally as ventricular premature complexes. A conservative approach with follow up was adopted in the case.

Giant congenital left ventricular diverticulum associated with infective endocarditis: A diagnosis made by tissue Doppler echocardiography

Left ventricular (LV) diverticulum is a relatively rare condition, and it is important to differentiate it from pseudoaneurysm. The increasing use of noninvasive imaging modalities can help to demonstrate different types of ventricular outpouching structures. We report a case of congenital LV diverticulum that is much larger than the usual size and is diagnosed with tissue Doppler echocardiography and cardiac magnetic resonance imaging. Although a ventricular diverticulum is mostly asymptomatic, in the case of this particular patient, it has become complicated with infective endocarditis.<Learning objective: Congenital ventricular diverticulum is a rare finding and can be diagnosed with noninvasive imaging modalities such as tissue Doppler echocardiography and cardiac magnetic resonance imaging rather than surgery. Although congenital ventricular diverticulum is asymptomatic in most cases, infective endocarditis is a known complication.>

Cardiac troponin T is necessary for normal development in the embryonic chick heart.

The heart is the first functioning organ to develop during embryogenesis. The formation of the heart is a tightly regulated and complex process, and alterations to its development can result in congenital heart defects. Mutations in sarcomeric proteins, such as alpha myosin heavy chain and cardiac alpha actin, have now been associated with congenital heart defects in humans, often with atrial septal defects. However, cardiac troponin T (cTNT encoded by gene TNNT2) has not. Using gene‐specific antisense oligonucleotides, we have investigated the role of cTNT in chick cardiogenesis. TNNT2 is expressed throughout heart development and in the postnatal heart. TNNT2‐morpholino treatment resulted in abnormal atrial septal growth and a reduction in the number of trabeculae in the developing primitive ventricular chamber. External analysis revealed the development of diverticula from the ventricular myocardial wall which showed no evidence of fibrosis and still retained a myocardial phenotype. Sarcomeric assembly appeared normal in these treated hearts. In humans, congenital ventricular diverticulum is a rare condition, which has not yet been genetically associated. However, abnormal haemodynamics is known to cause structural defects in the heart. Further, structural defects, including atrial septal defects and congenital diverticula, have previously been associated with conduction anomalies. Therefore, to provide mechanistic insights into the effect that cTNT knockdown has on the developing heart, quantitative PCR was performed to determine the expression of the shear stress responsive gene NOS3 and the conduction gene TBX3. Both genes were differentially expressed compared to controls. Therefore, a reduction in cTNT in the developing heart results in abnormal atrial septal formation and aberrant ventricular morphogenesis. We hypothesize that alterations to the haemodynamics, indicated by differential NOS3 expression, causes these abnormalities in growth in cTNT knockdown hearts. In addition, the muscular diverticula reported here suggest a novel role for mutations of structural sarcomeric proteins in the pathogenesis of congenital cardiac diverticula. From these studies, we suggest TNNT2 is a gene worthy of screening for those with a congenital heart defect, particularly atrial septal defects and ventricular diverticula.

Isolated congenital cardiac diverticulum originating from the left ventricular apex: Report of a pediatric case

Congenital ventricular diverticulum is a rare cardiac anomaly defined as a localized protrusion of the ventricular free wall. Although, it is usually asymptomatic, complications such as embolism, infective endocarditis, and arrhythmias can occur. The diagnosis can be made by echocardiography, cardiac magnetic resonance imaging, or catheter angiography. Surgical resection is the treatment of choice in symptomatic patients, whereas the management of asymptomatic patients often represents a therapeutic dilemma. We report here, a 9-month-old patient with asymptomatic congenital left ventricular (LV) diverticulum associated with epigastric hernia.

Abstract 18260: Familial Beals- Hecht Syndrome Associated With Bicuspid Aortic Valve and Right Ventricular Apical Diverticulum. An Unusual Combination of Congenital Defects Diagnosed in Adulthood by Multimodality Imaging

Introduction: Congenital contractural arachnodactyly (CCA) is an autosomal dominant disorder. CCA is phenotypically similar to Marfan syndrome, typically not affecting the aorta or eyes. Right ventricle (RV) diverticulum with bicuspid aortic valve (BAV) and CCA is not described. Case: 48 year-old caucasian female: cardiac apical aneurysm, scoliosis, BAV, and diagnosis of CCA. CCA affected her mother, brother, and daughter. PE: 5’ 7", 128 pounds, scoliosis, arachnodactyly, wing span of 69”, Ghent Nosology score 5. Cardiac exam: unremarkable. CT chest: contrast: a 2.7 x 2.2 cm arterially enhancing thin-walled lobular structure at the cardiac apex communicating with RV. Echo: RV apical pouch: 2.2 x 1.7 cm, narrow neck - mild systolic expansion. RV size, function and strain: normal. BAV with trace AR. Ascending aorta: 3.5 cm. LVEF 73%. Cardiac MRI: 2 x 2 x 2.5 cm diverticulum arising from the RV apex, 5 mm neck communicating with the aneurysm and RV. No delayed enhancement of the myocardium/ RV diverticulum. Gene testing: 16 genes associated with familial thoracic aortic aneurysm and dissection tested. Patient: heterozygous for a novel D1532N variant in the FBN2 gene. This variant is not reported as a mutation or benign polymorphism. Discussion: CCA associated with BAV and RV diverticulum is not described. CCA is genetically linked to FBN2. Congenital ventricular diverticulum is a rare finding, very infrequently affecting the RV. Multimodality imaging and gene testing may lead to better identification of this malformation and its association with genetic diseases. There is no standardized treatment of RV diverticulum. This asymptomatic patient received conservative management.

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Congenital Ventricular Crypt, Diverticulum or Aneurysm? A Finding to be Considered

Congenital Ventricular Crypt, Diverticulum or Aneurysm? A Finding to be Considered

Congenital ventricular diverticulum and MI – Diagnostic challenges and implications

The use of modern cardiac imaging techniques suggests that congenital ventricular diverticulum (CVD) may be more common than generally believed and may present asymptomatically in adult life. We present a case of congenital left ventricular diverticulum diagnosed in a patient presenting with myocardial infarction (MI). The case highlights the importance of the differential diagnosis of CVD from post infarct left ventricular aneurysms (PILVA) and suggests that adult studies using modern imaging techniques are needed to define the prognosis for asymptomatic CVD in order to guide management.