A Rare Case of Double-Chambered Right Ventricle Associated with Ventricular Septal Defect and congenital Absence of the Pulmonary Valve

Khoueiry, Georges; Bhat, Tariq; Tantray, Mohmad; Meghani, Mustafain; Rafeh, Nidal Abi; Abdallah, Marie; Hoyek, Wissam

doi:10.4137/ccrpm.s11174

Cited by 3 publications

(5 citation statements)

References 2 publications

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“…Double-chambered right ventricle is a rare congenital heart disorder involving 2 different RV pressure-compartments that is often associated with ventricular septal defect (VSD) [1,2]. In our case we could not find VSD.…”

Section: Discussionmentioning

confidence: 35%

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Przypadek dwujamowej prawej komory rozpoznanej na podstawie badania metodą rezonansu magnetycznego i cewnikowania

et al. 2017

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A double-chambered right ventricle (DCRV) is a heart defect, typically congenital, in which the right ventricle is separated into a proximal high-pressure (anatomically lower) chamber and distal low-pressure (anatomically higher) chamber. Commonly ventricular septal defect is found concomitantly in patients with DRCV. In this case report, we present a 20-year--old female patient who was found to have a DRCV without any concomitant congenital heart disorder.

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Section: Discussionmentioning

confidence: 35%

“…In our case we could not find VSD. Usually, the obstruction is caused by an anomalous muscle bundle crossing the RV from the interventricular septum to the RV free wall [1]. Because of its evolving nature, its diagnosis is usually made during childhood/adolescence and very rarely during adulthood [3].…”

Section: Discussionmentioning

confidence: 99%

Przypadek dwujamowej prawej komory rozpoznanej na podstawie badania metodą rezonansu magnetycznego i cewnikowania

et al. 2017

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“…Pulmonary valvular stenosis, ASD, aortic or tricuspid valve regurgitation, persistent left superior vena cava, ruptured sinus of valsalva aneurysm, and Ebstein anomaly are less common associations (4). Pulmonary valve agenesia, main pulmonary artery stenosis have also been reported (1,2).…”

Section: Discussionmentioning

confidence: 96%

“…An abnormal hypertrophic muscle band divides the right ventricle (RV) into two chambers (1). The defect between two chambers has a pressure gradient relative to the diameter (2). Although DCRV is usually asymptomatic, a progressive course (chest pain, heart failure symptoms) may occur.…”

Section: Introductionmentioning

confidence: 99%

“…Although DCRV is usually asymptomatic, a progressive course (chest pain, heart failure symptoms) may occur. Ventricular septal defect (VSD) is the most associated congenital heart defect at the rate of 80-90% (2). In the absence of a co-existing defect, surgery is not indicated unless the intracavitary systolic pressure gradient is higher than 40 mmHg or the obstruction is progressive to maintain normal RV function (1).…”

Section: Introductionmentioning

confidence: 99%

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Mid-term Results of Double Chamber Right Ventricle in Association with Genetic Syndromes

İştar,

Harmandar

2024

Muğla Sıtkı Koçman Üniversitesi Tıp Dergisi

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Objective Double chamber right ventricle (DCRV) is a congenital heart disease inwhich a hypertrophied muscle band divides the right ventricle (RV) chamber into two. The pressure gradient across the hole between two chambers is related to the diameter. Generally, DCRV is diagnosed in childhood, however in literature there are a few cases diagnosed in adulthood. Associations of DCRV and genetic syndromes had been published previously. Here we report early and mid-term follow-up of 6 patients with DCRV and distant genetic syndromes. Patients and Methods A retrospective analysis of 6 patients with DCRV (3 females and 3 males) in terms of age, weight, echocardiograhy, associated genetic syndromes was performed using data collected from the database of two institutions between 2016-2022. The mean age was 3.9 ± 1.4 years (2.5-5.5), mean weight was 12.1 ± 3.4 kg (8.7-15.5). The mean pressure gradient between proximal and distal chamber in RV was 42 ± 25 (17-67) mmHg. Concomitant cardiac anomalies were as follow: perimembranous ventricular septal defect (VSD) (3), large secundum atrial septal defect (1), aortic valve prolapse and/or mild aortic regurgitation (1), discrete subaortic membrane (1). Associated genetic syndromes were Costello Syndrome (1), Seckel Syndrome (1), Down Syndrome (4). Total correction was performed under cardiopulmonary bypass (CPB) using right atrial approach for all patients. The mean follow-up period was 4.86 ± 4.6 (0.26-9.46) years. Mean systolic pressure gradient in right ventricle at postoperative early follow-up period was 18.5 ± 11.5 (7-30) mmHg. Postoperatively the patient with Costello Syndrome had atrial fibrillation on 5th postoperative day and was treated with amiodarone infusion. There was no death or surgical reintervention during the mid-term follow-up period. Conclusion Early and mid-term surgical outcomes of DCRV are favorable. Surgical treatment of DCRV via right atriotomy can reduce the duration of intensive care unit stay and inotropic support. Additionally, DCRV can be diagnosed with different genetic syndromes like Costello Syndrome (1), Seckel Syndrome (1), Down Syndrome (4).

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Anomalous Muscle Bundles Causing Subpulmonic Stenosis

Chamanian

Jalalyazdi

Esfehani

et al. 2015

Jentashapir J Health Res

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Introduction: Muscular subpulmonic stenosis causing right ventricular outflow tract obstruction is an uncommon cardiac disease, especially when first diagnosed in adults. Diagnosis can be challenging when it is minimally symptomatic. Case Presentation: We report a 40-year-old man who was referred to cardiology clinic for work-up of dyspnea and a heart murmur found on his physical examination. A transesophageal echocardiogram (TEE) revealed a muscular subpulmonic stenosis and a small perimembranous ventricular septal defect (VSD) with left-to-right shunt and severe right ventricular outflow tract obstruction (RVOT). Right heart catheterization showed an estimated gradient of 75 mmHg across the two chambers due to subpulmonary stenosis. The pressure in the proximal right ventricular chamber was 100/25 mmHg and 25/17 mmHg in the distal chamber. Surgical correction was successfully performed. A postoperative TEE showed no residual gradients across the RVOT. Conclusions: Full echocardiographic examination of the right ventricle, including RVOT is essential for the diagnosis of this disease. Surgical correction appears to be a successful strategy for management of this rare condition in adults.

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A Rare Case of Double-Chambered Right Ventricle Associated with Ventricular Septal Defect and congenital Absence of the Pulmonary Valve

Cited by 3 publications

References 2 publications

Przypadek dwujamowej prawej komory rozpoznanej na podstawie badania metodą rezonansu magnetycznego i cewnikowania

Przypadek dwujamowej prawej komory rozpoznanej na podstawie badania metodą rezonansu magnetycznego i cewnikowania

Mid-term Results of Double Chamber Right Ventricle in Association with Genetic Syndromes

Anomalous Muscle Bundles Causing Subpulmonic Stenosis

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