Supracristal Ventricular Septal Defect Research Articles

Supracristal ventricular septal defect versus sinus of Valsalva aneurysm – a diagnostic dilemma solved on computed tomographic angiography

Supracristal ventricular septal defect versus sinus of Valsalva aneurysm – a diagnostic dilemma solved on computed tomographic angiography

Abstract 12143: Hearing is Believing: A Case of a Ruptured Sinus of Valsalva Aneurysm

A 36-year-old previously active and healthy male was referred to our Adult Congenital Heart Disease clinic with worsening exertional dyspnea and intermittent palpitations over the past 6 months. He had recently been diagnosed with a membranous ventricular septal defect (VSD) and membranous septal aneurysm at another hospital, although images were not available for review.On physical exam, a continuous cardiac murmur was noted which prompted reconsideration of the diagnosis, with highest suspicion of ruptured sinus of Valsalva aneurysm (SVA). An urgent cardiac CT was obtained, which showed a large, mobile right SVA protruding into and partially obstructing the right ventricular outflow tract (RVOT), with pulmonic regurgitation and dynamic closure of a supracristal VSD (Figure 1A). A margin of the SVA was ill-defined and suspicious for rupture, although no definite communication was seen. An expedited TTE showed findings consistent with SVA rupture (Figure 1B), and he was admitted for surgery soon thereafter. Intra-operatively (Figure 1C), SVA rupture into the RVOT was confirmed by direct inspection; the SVA tissue was resected, the defect closed with a fresh autologous pericardial patch, and the VSD was closed with pledgeted sutures. Post-operatively, there was no leakage around the patch margin and only trace aortic regurgitation.A ruptured SVA causes continuous shunting from the aorta into an adjacent cardiac chamber and if left unrecognized, can lead to right heart failure, arrhythmia, and potentially sudden death, emphasizing the need for early identification. Echocardiography may show an aneurysmal sinus of Valsalva with a continuous (with accentuation in diastole) high-velocity, turbulent flow-jet into an adjacent cardiac chamber, and holodiastolic aortic flow reversal; a VSD is often present. Cross-sectional imaging is also important for diagnosis and surgical planning. A comprehensive physical exam is crucial to mitigate the risk of misdiagnosis.

A Positive Microbubble Study in Ventricular Septal Defect Patient Without Pulmonary Hypertension

A stroke patient with supracristal ventricular septal defect accompanying suspected patent foramen ovale underwent transthoracic and transesophageal echocardiography with agitated saline microbubble study, a positive trans-ventricular microbubble jet after Valsalva maneuver provocation was detected, indicating instantaneous transient paradoxical right-to-left shunt at late diastole might be the etiology of embolic events. (Level of Difficulty: Intermediate.)

Doubly Committed Subarterial Ventricular Septal Defect Coexisted with Ruptured Aneurysm of The Right Sinus Valsava

Sinus of Valsalva aneurysms (SVAs) are uncommon cardiac abnormalities and most of them are in congenital origin. Some patients may have SVAs that concomitant with ventricular septal defect (VSD). Here, we reported case of a 37-year-old male presented with worsening exertional dyspnoea. Thrills and loud continuous murmur along the left sternal border were discovered during physical examination. Echocardiogram displayed ruptured sinus of valsalva aneurysm to the right ventricle along with supracristal ventricular septal defect (VSD). The patient underwent surgical correction with patch repair and aortic valve replacement. Most cases of SVAs were originated from right coronary sinus. They usually ruptured into right ventricle. Ventricular septal defects often coexisted with this condition. Echocardiography provided a complete evaluation of such cases and prohibited missed diagnosis of other coexistent congenital heart defects.

Adult Supracristal Ventricular Septal Defect with Aortic Regurgitation: A Case Report

Supracristal Ventricular Septal Defect (VSD) develops Aortic Regurgitation (AR) with the passage of time due to prolapse of the right coronary cusp. It brings clinical attention in early child hood with an incidental continuous murmur, recurrent respiratory tract infection of failure to thrive. This report is about a rare case of 52-year-old female who presented with increased fatiguability since the last six months. Clinical examination revealed the presence of cardiomegaly with holosystolic-diastolic murmur in Left Upper Sternal Border (LUSB). Echocardiography revealed the presence of supracristal VSD with moderate AR without prolapsing aortic leaflet or dilatation of aortic sinus. In view of presence of moderate AR, the patient was advised to be on Angiotensin Receptor Blocker (ARB) with regular follow-up to monitor the progression of AR. Such a late index presentation of supracristal VSD with moderate AR in fifth decade of life has not been described in literature so far. To conclude, nature retards the natural history with bless in disguise.

A challenging case report of acute sinus of Valsalva aneurysm rupture in cardiogenic shock and multi-organ failure with an emphasis on rapid recognition and ‘never giving up’ in face of futility

Background Sinus of Valsalva aneurysm (SoVA) is a rare anomaly and can be divided into acquired and congenital forms, the latter being commonly associated with ventricular septal defects (VSDs). Rupture is a catastrophic complication with high mortality without urgent surgical intervention. We would like to highlight the use of echocardiography in an emergency setting for diagnosis and surgical intervention in a critically ill patient.Case summary We report a 27-year-old female with history of conservatively managed VSD known since childhood. She presented with acute decompensated cardiac failure requiring intubation and inotropic support. Bedside echocardiography performed in the emergency department suggested a ruptured SoVA at the right coronary cusp with underlying supracristal VSD. Despite the patient being critically ill with multi-organ failure, surgery was performed as it was the patient’s best chance for survival. Intraoperative findings tallied with the early echocardiographic results. She recovered gradually and was eventually discharged despite a stormy post-operative period.Discussion This case report highlights the importance of prompt recognition of SoVA rupture by using bedside echocardiography. Surgical intervention needs to be early despite ongoing sepsis in view of acute mechanical failure. This case was unique as it illustrates a successful management of an acutely ill patient with multi-organ failure through early diagnosis, intensive perioperative stabilization, and surgical intervention.

Aberrant right coronary artery in a grown up congenital cardiac patient, successfully treated 46 years earlier with a double Starr-Edwards silastic ball valve replacement: a case report

BackgroundThe Starr-Edwards ball valve prosthesis was successfully introduced in 1961–62 and largely used for aortic and mitral valve replacement. Even if Starr-Edwards valves have been widely replaced in clinical practice by other mechanical valves, they define a standard concerning long-term durability.Case presentationWe describe the case of a 55-year-old man referred to our Department to perform a cardiac computed tomography (CCT), to better evaluate a severe dilation of ascending aorta discovered at echocardiography. The patient had been surgically treated 46 years earlier to correct a supra-cristal type ventricular septal defect. Both mitral and aortic valves were replaced, respectively due to bacterial mitral endocarditis and a fibrous sub-valvular aortic stenosis. In addition, the right coronary artery (RCA) was found to arise from the left coronary sinus.ConclusionWe report the longest lasting durability (46 years) of aortic and mitral Starr-Edwards valves successfully implanted in a patient simultaneously carrying a malignant anomalous origin of RCA.

507 Heat failure in restrictive ventricular septal defect

Abstract Introduction Patients with small restrictive ventricular septal defect are usually asymptomatic. This case report presented a case of shortness of breath in a patient with known restrictive ventricular septal defect. Case report A 51 years old woman was diagnosed to have a small supra-cristal ventricular septal defect in early twenties. She enjoyed good functional state since the diagnosis. Routine echocardiogram assessment one year ago found normal left ventricular size and systolic function. However, she presented with exertional shortness of breath and severe bilateral lower limbs edema for two weeks. Transthoracic echocardiogram found both left and right ventricles were dilated. Left ventricular ejection fraction was 50%. The right ventricular systolic pressure(RVSP) was 70mmHg from tricuspid regurgitation peak velocity estimation. It was significantly raised compared with the RVSP 35mmHg measured last year. This caused right ventricular pressure overload with systolic flattening of interventricular septum. On color doppler examination, apart from the known ventricular septal defect flow, there was an abnormal turbulent flow at the right ventricular outflow tract. The two jets was close to each other and the nature of the abnormal jet could not be clearly identified. During trans-esophageal echocardiogram, in order to differentiate the two different jets, the baseline of the color doppler was shifted towards the directions of the jets to look for the proximal isovelocity surface area (PISA). Finally there were two PISA could be clearly seen. One was the PISA of the ventricular septal defect while the other one was due to ruptured right coronary sinus with shunting from aorta to right ventricular outflow tract. The findings was supported by continuous wave doppler examination. The ventricular septal defect flow was predominantly systolic whereas the aorto-right ventricular shunting was a continuous flow. Moreover, three dimension echocardiogram also showed the two closely related holes. Finally the patient underwent percutaneous closure of the ruptured sinus of valsalva. And the patient recovered well afterwards. Discussion Small ventricular septal defect with restrictive physiology usually will not lead to heart failure. When patient presented with heart failure while having a small ventricular septal defect, other pathology should be carefully looked for. Ruptured sinus of valsalva was known to associate with ventricular septal defect. However, the jet of ruptured sinus of valsalva may be missed in view of the close proximity with the ventricular septal defect jet as in this case. Methods to better delineate the different jets including demonstration of double PSIA, continuous flow on continuous wave doppler and three dimension echocardiogram. Nowadays, ruptured sinus of valsalva could also be closed percutaneously with success. Abstract 507 Figure.

Obesity in Patient with Grown Up Congenital Heart Disease Ventricular Septal Defect with Pulmonary Hypertension in Sanglah Hospital, Bali, Indonesia

Ventricular septal defect (VSD) is a congenital heart disease (CHD) which characterized bya hole in the wall that separate between the right and left ventricle. There are several typesof VSD based on its location, which are perimembranous/conoventricular, subpulmonary/conotruncal, supracristal/doubly commited, muscular and atrioventricular canal/inlet. AVSD is one of the most common CHD which occurs in approximately 2-6 of every 1,000live births and accounts for more than 20% of all CHDs. However, the population of VSDpatients with grown up congenital heart disease (GUCH) is still uncertain. We are presentinga rare case of a 19 year old patient with congestive heart failure NYHA functional class IVdue to uncorrected left-to-right shunt supracristal VSD with body mass index of 35.5 kg/m2. He presented in the emergency room with acute decompensated heart failure profileB. Echocardiography revealed decreased left ventricle (LV) systolic function, normal rightventricle (RV) systolic function, decreased LV diastolic function, and dilated left atriumand LV. We plan to perform right heart catheterisation and pulmonary artery oxygenresponsiveness test followed by VSD closure in this patient. Another explanation will bediscussed in this paper.

Recent advances in managing septal defects: ventricular septal defects and atrioventricular septal defects.

This review discusses the management of ventricular septal defects (VSDs) and atrioventricular septal defects (AVSDs). There are several types of VSDs: perimembranous, supracristal, atrioventricular septal, and muscular. The indications for closure are moderate to large VSDs with enlarged left atrium and left ventricle or elevated pulmonary artery pressure (or both) and a pulmonary-to-systemic flow ratio greater than 2:1. Surgical closure is recommended for large perimembranous VSDs, supracristal VSDs, and VSDs with aortic valve prolapse. Large muscular VSDs may be closed by percutaneous techniques. A large number of devices have been used in the past for VSD occlusion, but currently Amplatzer Muscular VSD Occluder is the only device approved by the US Food and Drug Administration for clinical use. A hybrid approach may be used for large muscular VSDs in small babies. Timely intervention to prevent pulmonary vascular obstructive disease (PVOD) is germane in the management of these babies. There are several types of AVSDs: partial, transitional, intermediate, and complete. Complete AVSDs are also classified as balanced and unbalanced. All intermediate and complete balanced AVSDs require surgical correction, and early repair is needed to prevent the onset of PVOD. Surgical correction with closure of atrial septal defect and VSD, along with repair and reconstruction of atrioventricular valves, is recommended. Palliative pulmonary artery banding may be considered in babies weighing less than 5 kg and those with significant co-morbidities. The management of unbalanced AVSDs is more complex, and staged single-ventricle palliation is the common management strategy. However, recent data suggest that achieving two-ventricle repair may be a better option in patients with suitable anatomy, particularly in patients in whom outcomes of single-ventricle palliation are less than optimal. The majority of treatment modes in the management of VSDs and AVSDs are safe and effective and prevent the development of PVOD and cardiac dysfunction.

Supracristal ventricular septal defect in adults: Is it time for a paradigm shift?

BackgroundTo determine the risk and predictors of progression of aortic regurgitation (AR) and other adverse events (AE) in adults with supracristal ventricular septal defect (VSD). MethodsRetrospective observational study of 62 adults with supracristal VSD followed at the Mayo Clinic from 1994 to 2013. Freedom from AR progression was compared by age and racial groups (Asian vs non-Asian). Predictors of AR progression were determined using Cox proportional hazard model. Composite AE endpoint was defined as AR progression, endocarditis, aneurysm of sinus of Valsalva (aSOV), and rupture of aSOV. Risk of AE was compared between group 1 (no surgical intervention) and group 2 (surgical intervention). ResultsSixty-two patients aged 47(SD 12) years were followed for 13 (SD 5) years. Group 1=42/62 and group=20/62. Endpoint of AR progression and aSOV occurred in 13% and 20% respectively. Freedom from AR progression for the entire cohort was 88%, 86% and 86% at 5, 10 and 15years respectively. Risk of AR progression was significantly lower in patients (aged ≤40years, p=0.008) but similar between Asians and non-Asians (p=0.57). Age ≤40years was an independent predictor of AR progression (hazard ratio [HR] 3.5, 95% CI 2.3–5.4, p=0.001). Composite AE endpoint occurred in 33% and 40% of group1 and 2 cohorts respectively, p=0.69. ConclusionAdults with supracristal VSD are at lower risk for AR progression but higher risk for aSOV formation compared to historical pediatric cohorts. Younger age was a predictor of AR progression. Surgical intervention had no protective effect on complication rate.

Surgical treatment following transcatheter intervention in an adult patient with supravalvar mitral ring, large patent ductus arteriosus and severe pulmonary hypertension

Supravalvar mitral ring (SVMR) is a rare cause of congenital mitral stenosis. It can occur in isolation but often coexists with other cardiac anomalies [1]. Conversely, a patent ductus arteriosus (PDA) is a much more common anomaly. In terms of cardiac hemodynamics, pulmonary arterial hypertension (PAH) can occur if there is left-sided obstruction by SVMR or high pulmonary blood flow by PDA. But the coexistence of both mechanisms is very rare. We herein introduce a case of a 23-year-old female with SVMR, a large PDA, supracristal ventricular septal defects (SPVSD) and severe PAH.

Prognostic Implications of Initial Echocardiographic Findings in Adolescents and Adults with Supracristal Ventricular Septal Defects

Although surgery is recommended for pediatric patients with supracristal ventricular septal defects (sVSDs) to prevent progression of aortic regurgitation (AR), outcomes in adolescents and adults with sVSDs are not known. In this retrospective observational study, clinical data without surgery were obtained in 60 patients with sVSDs (group 1; mean age, 36 ± 13 years), 120 age- and defect size-matched patients with perimembranous ventricular septal defects (group 2), and 52 patients with sVSDs who underwent surgery (group 3; mean age, 32 ± 11 years). Aortic sinus wall prolapse (38% vs 3%, P < .0001) and moderate to severe AR (7% vs 0%, P= .012) were more frequently observed in group 1 than in group 2. Five, three, and two patients in group 1 had surgery during follow-up because of rupture of the aneurysm of the sinus of Valsalva, endocarditis, and heart failure, respectively. Group 1 had a lower 12-year clinical event-free (surgery and endocarditis) rate (76 ± 9% vs 94 ± 4%, P= .031) but an equivalent overall survival rate (100% vs 94 ± 3%, P= .143) compared with group 2. Patients with maximal prolapsing aortic sinus wall length > 7 mm showed a higher frequency of aneurysm of the sinus of Valsalva rupture than those with no prolapse or maximal prolapsing length ≤7mm (80% [four of five] vs 2% [one of 55], P< .001). The event-free and overall survival rates were comparable between groups 1 and 3, with equivalent 10-year AR progression-free survival rates (94 ± 5% vs 91± 5%, P= .301). Aneurysm of the sinus of Valsalva rupture, rather than AR progression, was the main clinical event. Watchful monitoring of patients with high-risk echocardiographic features may be a rational option.

Laubry-Pezzi Syndrome With Aortic Root Dilatation Treated With a Bentall and De Bono Procedure

Laubry-Pezzi syndrome is a clinical entity in which prolapse of an aortic valve cusp into a subjacent ventricular septal defect (VSD) due to Venturi effect results in progressive aortic valve insufficiency. Aortic valve prolapse is found in over 5% of children with VSDs, most commonly in association with supracristal VSDs, and the risk of development of aortic regurgitation increases during childhood, peaking at 5 to 10 years of age. The VSD closure eliminates the low-pressure zone that is the cause of ongoing aortic valve cusp deformity and, if performed early, prevents the development of aortic regurgitation. However, the management of this rare pathology is still a matter of some debate with respect to indications, operative techniques, and timing. We report the case of a patient with Laubry-Pezzi syndrome, originally operated on at six years of age for VSD closure and plasty between the left and the right aortic cusps. However, in the follow-up the patient developed severe aortic regurgitation and aortic root dilatation. Eventually, a Bentall and De Bono procedure was performed.

Aortic insufficiency due to ventricular septal defect (Laubry–Pezzi syndrome)

A 15-year-old boy showed progressive aortic valve regurgitation during follow-up of supracristal ventricular septal defect. This anatomic arrangement is consistent with the Laubry-Pezzi syndrome in which prolapse of a non-coronary aortic valve cusp due to Venturi's effect results in progressive aortic valve insufficiency.

Surgery for Ruptured Sinus of Valsalva Aneurysm into Right Ventricular Outflow Tract: Role of Intraoperative 2D and Real Time 3D Transesophageal Echocardiography

A major limitation of the 2D echocardiography during surgery for a complex cardiac lesion is its inability to provide an accurate spatial orientation of the structure. The real time 3D transesophageal echocardiography (RT-3D-TEE) technology available in Philips IE 33 ultrasound machine is relatively new to an operation suite. We evaluated its intraoperative utility in a patient, who was operated for repair of a ruptured sinus of Valsalva aneurysm (RSOVA) and closure of a supracristal ventricular septal defect. The VSD and RSOVA were visualized through different virtual windows in a more promising way on intraoperative RT-3D-TEE than on the 2D echocardiography. The acquired images could be virtually cropped and displayed in anatomical views to the operating surgeon for a clear orientation to the anatomy of the lesion. RT-3D-TEE is a potential intraoperative monitoring tool in surgeries for complex cardiac lesions.

Double Chamber Right Ventricle in a Patient With Supracristal Ventricular Septal Defect and Prolapsing Right Coronary Cusp: Role of Intraoperative Transesophageal Echocardiography

A supracristal ventricular septal defect (VSD), an outlet VSD situated in subaortic and subpulmonic regions, has a strong association with prolapse of the aortic valve cusp. The authors report the case of a patient operated for a supracristal VSD with prolapse of the right coronary cusp. The VSD was found to be in the subaortic position on preoperative transthoracic echocardiography, which failed to detect the presence of a double-chamber right ventricle. Intraoperative transesophageal echocardiography correctly recognized the supracristal nature of the VSD and identified the double-chamber right ventricle, subsequently altering the course of surgery.

A multi-perforated man: Asymptomatic ruptured sinus of Valsalva aneurysm associated with an atrial and ventricular septal defect

We report the case of an exceptional association of a right coronary sinus of Valsalva aneurysm (SVA) ruptured into the right ventricle, a supracristal ventricular septal defect (VSD) and an atrial septal defect (ASD). Our patient was totally asymptomatic and the diagnosis was established by echocardiography. The patient underwent prompt surgery that consisted in closing the aneurysm and the VSD with a pericardium patch.

Surgical correction of ventricular septal defect with aortic regurgitation in a dog

An 8-month-old entire Miniature Dachshund, weighing 4.2 kg, was presented for examination following delvelopment of a cough. Ventricular septal defect had been diagnosed tentatively in its infancy on the basis of a cardiac murmur detected by auscultation and echocardiography. Echocardiography using a B mode right parasternal long-axis view showed a defect at the atrioventricular junction and a thickened cusp of the aortic valve prolapsing into the defect. Colour-flow Doppler showed shunt blood flow across the defect at the level of the atrioventricular junction, from left to right. The sinus of Valsalva was dilated, with turbulent blood flow. Aortic regurgitation was also observed. Cardiac catheterisation studies confirmed the diagnosis of a supracristal ventricular septal defect with aortic regurgitation. Despite medication with digoxin, enalapril and aminophylin, started from the first admission, left ventricular internal dimensions gradually increased, and fractional shortening of the left ventricle gradually decreased. Surgery, with the aid of extracorporeal circulation, to close the ventricular septal defect, was performed 1 year after the initial examination. The aortic valve was left untreated. Postoperatively, the systolic murmur disappeared. Shunt flow from the left to the right ventricle was no longer observed on echocardiography, however there was still a small amount of aortic regurgitation during diastole visualised with colour-flow Doppler echocardiography. The prolapse of the cusp of the aortic valve on B-mode echocardiography was no longer observed and thickening of the cusp had not progressed. Left ventricular function measurement using M mode echocardiography showed a reduced left ventricular volume overload with reduced left ventricular internal dimensions and increased fractional shortening. The cough was relieved and no follow-up medication was scheduled. Early surgical closure of the ventricular septal defect improved the patient's condition and controlled prolapse and thickening of the aortic valve.

Percutaneous transcatheter coil embolization of a ventricular septal defect in a dog

A 4-month-old male French Bulldog weighing 5.0 kg (11 lb) was referred for a heart murmur. A grade 3/6 systolic murmur was detected at the left heart base and a grade 4/6 systolic murmur was detected at the right heart base. By use of color-flow Doppler ultrasonography and cardiac catheterization, a diagnosis of supracristal ventricular septal defect (VSD) with accompanying aortic regurgitation was made. Percutaneous transcatheter coil embolization was used to close the VSD. Because residual shunt was detected via echocardiography after coil implantation, the residual shunt was followed periodically via echocardiography to detect spontaneous closure of the VSD. Volume overload in the left ventricle was detected in the dog 131 days after admission. Additional coils were placed 137 days after admission. Hemolysis resulting in hemoglobinuria was detected, but this complication was mild. In the dog of this report, results of coil occlusion for correction of VSD were promising. Thus, coil occlusion should be considered as an alternative treatment for VSD in dogs.