Restrictive Ventricular Septal Defect Research Articles

Transcatheter Ventricular Septal Defect (VSD) Creation for Restrictive VSD in Double-Outlet Right Ventricle

BackgroundDouble-outlet right ventricle (DORV) with a restrictive ventricular septum is a rare but highly morbid phenomenon that can be complicated by progressive left ventricular hypertrophy, arrhythmias, aneurysm formation, severe pulmonary hypertension, and death in the newborn. Surgical creation or enlargement of a ventricular septal defect (VSD) is palliative but may damage the conduction system or the atrioventricular valves in the newborn. This report presents a transcatheter approach to palliation for a newborn that had DORV with a restrictive ventricular septum.Methods/ResultsA full-term infant girl (2.9 kg) referred for hypoxia (80 % with room air) and murmur was found to have DORV, interrupted inferior vena cava, and restrictive VSD (95-mmHg gradient). Transhepatic access was performed, and an internal mammary (IM) catheter was advanced through the atrial septal defect and into the left ventricle. By transesophageal echocardiographic guidance, a Baylis radiofrequency perforation wire was used to cross the ventricular septum, and the defect was enlarged using a 4-mm cutting balloon. A bare metal stent then was deployed to maintain the newly created VSD. The patient did well after the procedure but required pulmonary artery banding 4 days later. She returned 5 months later with cyanosis and the development of obstructing right ventricle muscle bundles, requiring further surgical palliation.ConclusionsThis report describes the first transcatheter creation of VSD in DORV with a restrictive ventricular septum in a newborn infant. Use of the radiofrequency catheter in combination with cutting balloon dilation and stent implantation is an efficient method for creating a VSD in such a patient.Electronic supplementary materialThe online version of this article (doi:10.1007/s00246-012-0337-1) contains supplementary material, which is available to authorized users.

Aortic Translocation Using the Hemi-Mustard Procedure for Corrected Transposition

The management of congenitally corrected transposition of the great arteries and associated lesions is frequently challenging. Restrictive ventricular septal defect and mild pulmonary stenosis are contraindications to the double switch procedure, including the atrial-Rastelli switch procedure, due to the production of postoperative left ventricular outflow tract obstruction. We describe a case of aortic translocation using the hemi-Mustard procedure after left ventricular training in order to prevent postoperative left ventricular outflow obstruction.

Complicações da biópsia endomiocárdica após transplante cardíaco. Um mal menor

Endomyocardial biopsy is still the principal method for diagnosing cardiac allograft rejection. However, this procedure can be associated, albeit rarely, with potentially serious complications.We describe the case of a patient with extensive anterior myocardial infarction without revascularization, who developed cardiogenic shock and required heart transplantation. Post-transplantation, a coronary artery fistula to the right ventricle associated with an aneurysm and two restrictive ventricular septal defects were detected.

Modified Nikaidoh procedure in a patient with transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction with unusual coronary anatomy

The Rastelli operation has been the most common procedure for the repair of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction. A relatively recent approach is the Nikaidoh procedure. Despite the fact that it seems promising, the operation lacks long-term follow-up data. It has been postulated that patients with anomalous coronary arteries are high-risk candidates for the Nikaidoh procedure and its modifications. In this report, we present the case of a patient with transposition of the great arteries with remote restrictive ventricular septal defect and left ventricular outflow tract obstruction with coronary anomaly - with the right coronary artery originating from the left anterior descending coronary artery and crossing the right ventricular outflow tract - who underwent successful modified Nikaidoh operation.

Right Ventricular Endocarditis in a Pregnant Woman with a Restrictive Ventricular Septal Defect

A 22-year-old woman with a restrictive unoperated perimembranous ventricular septal defect was diagnosed with staphylococcal endocarditis during her 14th week of pregnancy. Echocardiography revealed a long, thin, and mobile vegetation along the right ventricular free wall that increased to 8 cm in length, with systolic protrusion across the pulmonary valve. The vegetation subsequently embolized, resulting in a pulmonary abscess. She responded favorably to intravenous antibiotic therapy maintained for a total of 6 weeks, with resolution of the intracardiac mass and pulmonary abscess. The remaining peripartum and postpartum course was relatively unremarkable. Percutaneous closure of the ventricular septal defect was successfully performed postpartum.

Tetralogy of Fallot With Restrictive Ventricular Septal Defect

We present a case of tetralogy of Fallot with progressive restriction of the ventricular septal defect (VSD) by tricuspid valve tissue. The report includes echocardiographic and catheter images of this rare complication of tetralogy of Fallot. Restriction of the VSD in tetralogy of Fallot is associated with increased surgical-related mortality.

Right ventricular remodeling in restrictive ventricular septal defect

Restrictive ventricular septal defect (rVSD) presents with little/no hemodynamic aberrations despite a patent septal defect. Clinically, these patients are observed with the hope that the defect will functionally close over time without the need for surgical repair and development of heart failure. Without evidence supporting a definitive therapeutic strategy, rVSD patients may have increased risk of a poor outcome. We tested the hypothesis that rVSD results in subclinical RV diastolic dysfunction and molecular remodeling. Five pigs underwent surgical rVSD creation. Echocardiography, hemodynamics, myocyte contractility experiments, and proteomics/Western blot were performed 6-weeks post-rVSD and in controls. * p < 0.05. LV and RV hemodynamics in rVSD were comparable to controls. The tricuspid valve early/late diastolic inflow velocity ratio (TV E/A ratio) decreased from 1.6 ± 0.05 in controls to 1.0 ± 0.08* in rVSD, indicating RV diastolic dysfunction. rVSD RV myocytes showed abnormalities in contraction (departure velocity (Vd) − 51%*, Vd time + 55%*) and relaxation (return velocity (Vr) − 50%*, Vr time + 62%*). Mitochondrial proteins (fatty acid, TCA cycle) increased 2-fold*, indicating heightened RV work. Desmin protein upregulated 285%* in rVSD RV myocardium, suggesting cytoskeletal remodeling. rVSD causes RV diastolic dysfunction, myocyte functional impairment, and mitochondrial/cytoskeletal protein upregulation in our model. Desmin upregulation may hinder sarcomeric organization/relaxation, representing a key subclinical early marker for future RV dysfunction. TV E/A measurements are a non-invasive modality to assess rVSD patients for diastolic dysfunction. Translational research applications may lead to fundamental changes in the clinical management of rVSD by providing evidence for early repair of the defect.

Muscular ventricular septal defect after mitral and aortic valve replacement

From September 2007 to December 2008, five patients aged 54–69 years were admitted to our institution for cardiac rehabilitation after surgical cardiac valve replacement (table 1). Successful replacement of the aortic valve was performed in four of the patients and mitral valve replacement in the other. All patients received mechanical prosthetic valves (ATS medical) and had a history of dyspnoea, fatigue and exercise limitation. Physical examination on admission did not reveal signs of congestion in any of the patients. The admission electrocardiogram showed sinus rhythm or atrial fibrillation (patient 4) and signs of left ventricular hypertrophy in 3 of 5 patients. Doppler echocardiography revealed a restrictive muscular ventricular septal defect (fig. 1, 3, 4, 5 and 6) with Qp/Qs <1.4 in all of the patients. The function of the prosthetic valves was normal and in one patient (patient 5) systolic pump function was moderately reduced. Echocardiographic follow-up revealed spontaneous closure of the septal perforation in four of the five patients (fig. 2). In one patient the septal defect persisted for one and a half years after surgery, but he remained asymptomatic without deterioration of left ventricular function or increased pulmonary artery systolic pressure.

Restrictive ventricular septal defect and critical subaortic stenosis in tetralogy of Fallot

Tetralogy of Fallot is characterized by a non-restrictive malalignment ventricular septal defect (VSD), an overriding aorta and right ventricular outflow tract obstruction resulting from anterior deviation of the infundibular septum. Due to the large VSD, systolic pressures are equal in both ventricles. In rare cases, redundant tricuspid valve tissue may prolapse into the VSD resulting in restriction of the defect size and causing suprasystemic pressure in the right ventricle. Subaortic obstruction by prolapse of the redundant tricuspid tissue into the left ventricular outflow tract, although theoretically possible in this situation, has not been described yet in the literature. We report on a newborn with tetralogy of Fallot presenting with cyanosis and severe arterial hypotension a few hours after birth. Echocardiography revealed redundant hammock-like accessory tricuspid valve tissue almost completely occluding the originally large VSD. Suprasystemic pressure in the right ventricle resulted in protrusion of tricuspid valve tissue across the VSD and thereby caused severe left ventricular outflow tract obstruction. Emergency pulmonary balloon valvuloplasty performed for decompression of the right ventricle, reduced prolapse of tricuspid tissue into the left ventricular outflow tract and resulted in significant improvement of pulmonary and systemic blood flow. In tetralogy of Fallot, accessory tricuspid valve tissue may obstruct the VSD as well as the left ventricular outflow tract resulting in a life threatening condition in newborns shortly after birth.

Elastin Arteriopathy and William Syndrome: Do You Feel Lucky?

To the Editor: Over the past 2 yr, we have anesthetized 10 patients with Williams syndrome and concur with the recommendations by Burch et al.1 in their recent article. In addition, we have additional suggestions based on the successful resuscitation of two infants who suffered a cardiac arrest under anesthesia. In both infants, induction of anesthesia was with incremental doses of sevoflurane (2% and 3%), a remifentanil infusion and rocuronium. Cardiac arrest occurred within 5 min of induction and before intubation. The electrocardiograms demonstrated marked ST segment depression that was followed soon after by bradycardia. Neither patient had discernible systemic output (no palpable or Doppler evidence of pulses). Resuscitation with phenylephrine, epinephrine, and small fluid boluses occurred during the institution of cardiopulmonary resuscitation. Within 5 min from the start of resuscitation, the transthoracic echocardiography (TTE) demonstrated biventricular akinesis in the first and severe biventricular hypokinesis in the second patient. We used TTE to guide our therapy, allowing us to monitor wall motion abnormalities, guide fluid administration, and carefully titrate vasopressors without worsening tachycardia or the dynamic component of subarterial outflow tract obstruction. In addition, we avoided administration of epinephrine once the rhythm returned, but pulses were still absent (pulseless electrical activity) because the TTE demonstrated severely hypertrophied, underfilled ventricles. In this population, the inappropriate administration of epinephrine as per the pediatric advanced life support protocol for pulseless electrical activity could have compromised recovery. The second patient required urgent extracorporeal membrane oxygenation cannulation after sustaining another cardiac arrest several hours after arrival in the pediatric intensive care unit within 3 min after receiving a 1 μg/kg dose of fentanyl. Both the patients underwent successful surgical repair. These patients had severe bilateral outflow track obstruction with what Stamm et al.2 have termed “macaroni pulmonary arteries” (Fig. 1). It is noteworthy that the presence of an intracardiac shunt (patent foramen ovale and small restrictive ventricular septal defect) did not decrease the risk of arrest.Figure 1.: The PA hypoplasia.Despite an induction technique designed to minimize changes in coronary perfusion, our experience demonstrates that these patients continue to have a very high-anesthetic risk of cardiac arrest. We postulate that, during induction, a decrease in coronary perfusion pressure occurred secondary to vasodilation and in combination with relative hypovolemia, and severe biventricular hypertrophy precipitated ischemia leading to cardiac arrest. In the second patient, a cardiac arrest occurred within minutes after a small dose of fentanyl, highlighting the precarious hemodynamic balance of these children. We recommend preinduction hydration, ideally with an IV, but at minimum liberal PO fluids until 2 h preinduction and the early use of TTE to help treat hemodynamic abnormalities and guide therapy. Denise C. Joffe, MD Michael Richards, BM Michael Eisses, MD Brian Emerson, MD Jeremy Geiduschek, MD Department of Pediatric Anesthesiology University of Washington Seattle, Washington [email protected]

Mid-term results of right axillary incision for the repair of a wide range of congenital cardiac defects☆

We evaluated the mid-term results of the right axillary incision used for the repair of various congenital heart defects. All the patients who were operated with this incision between March 2001 and December 2007 were reviewed. There were 123 patients (median age 4.7 {0.4-19.4} years and median weight 16.6 {3.8-62} kg) undergoing atrial septal defect (ASD) closure (62), repair of partial anomalous pulmonary venous connection (PAPVC) (22), correction of partial atrioventricular septal defect (AVSD) (19), and restrictive perimembranous ventricular septal defect (VSD) (20). Additional procedures involved tricuspid valve plasty (10), mitral annuloplasty (3), reduction plasty of the aortic sinus (2), resuspension of the aortic valve cusp (2), sub aortic membrane resection (1), or reimplantation of Scimitar vein (1). The surgical technique involved peripheral (groin) and central (SVC+/-aorta) cannulation for institution of cardiopulmonary bypass. Fibrillatory arrest was used for repair of ASDs and cardioplegic arrest for repairs involving the atrioventricular valves as well as VSDs. The median CPB and aortic clamp times were 72 (35-232) and 0 (0-126) min, respectively. There was no need for conversion to another approach in any patient. Early morbidity included transient paresis of left upper arm (1), stenting of SVC after repair of a sinus venosus defect (1) and revision for bleeding (1). Follow-up echo showed no residual defect in 116 patients and minor residual defects in 7 patients: tiny ASD (2), tiny VSD (1) and mitral regurgitation (4). One patient developed stenosis in the right external iliac artery used for cannulation, necessitating surgical intervention. All the patients are in excellent condition after a median follow-up of 4.1 (0.4-7.1) years. The incision healed well and the thorax and the breast showed no deformity on follow-up. The right axillary incision provides a quality of repair for various congenital defects similar to that obtained by using standard surgical approaches. Because of its deceitful location, and the camouflaging effect of being hidden by the resting arm, it has superior cosmetic appeal compared to conventional incisions. The incision does not interfere with subsequent development of the thorax or the breast (in case of females).

Spontaneous Regression of Left Ventricular Dilation in Children with Restrictive Ventricular Septal Defects

Spontaneous Regression of Left Ventricular Dilation in Children with Restrictive Ventricular Septal Defects

Nikaidoh operation for transposition of the great arteries with a ventricular septal defect and pulmonary stenosis

The optimal surgical management of patients with transposition of the great arteries with a ventricular septal defect and pulmonary stenosis remains challenging. When compared to other surgical options, the Nikaidoh procedure results in a 'more normal' anatomic result, with better alignment of the right and left ventricular outflow tracts. Also, the pulmonary outflow is less likely to be compressed by the sternum, a major issue associated with the Rastelli repair. This technique is especially useful in the presence of an inlet or restrictive ventricular septal defect, a hypoplastic right ventricle, a straddling atrioventricular valve and/or anomalous coronary anatomy interfering with a distal right ventricular outflow tract incision.

Unusual Hemodynamic Changes in an Infant with a Restrictive Ventricular Septal Defect

A 7-month-old asymptomatic infant was known to have a restrictive membranous ventricular septal defect partially closed by an aneurysm of the membranous septum. At 13 months of age, he developed unexpected pulmonary hypertension, with no clinical sign of cardiac failure. Cardiac catheterization assessed the pulmonary artery pressure at a systemic level with significant left-to-right shunt. After surgical closure, the pulmonary arterial pressure decreased by 50%. We suspect an enlargement of the ventricular septal defect caused by the rupture of the aneurysm of the membranous septum-a rare complication.

Spontaneous Regression of Left Ventricular Dilation in Children with Restrictive Ventricular Septal Defects

To test the hypothesis that left ventricular (LV) dilation associated with pressure-restrictive ventricular septal defect (VSD) often remains stable or regresses spontaneously, calling into question the role of interventional management for such defects. We analyzed 96 serial echocardiograms from 33 unoperated patients with a moderate-to-large VSD with LV dilation (LV end-diastolic dimension [LVED] z score >2.0) at enrollment who were followed for more than 2 years. Records of 125 surgical patients also were reviewed. Patients were evaluated for evidence of persistent or progressive LV dilation; signs or symptoms of congestive heart failure (CHF), failure to thrive (FTT), or pulmonary hypertension (PAH); as well as acquired ventricular outflow obstruction or aortic regurgitation. LVED z scores at enrollment versus latest follow-up were compared using paired t tests. A random-effects model with random intercept and slope was fitted to account for repeated observations for each patient. Mean age at enrollment was 4.6 +/- 3.2 years, and mean follow-up was 7.8 +/- 4 years (range, 2.8 to 22 years), during which mean LVED z score decreased from 3.0 +/- 0.6 to 1.2 +/- 1.3 (P < .01). LVED z score decreased in 29 of the 33 patients, and decreased to <2 in 26 of these 29 (79%). Most patients with pressure-restrictive VSD with moderate-to-severe LV dilation without CHF, FTT, or PAH will experience spontaneous resolution of LV dilation and can avoid cardiac surgery or catheter-based intervention.

Complex conotruncal cardiac anomalies consecutively in three siblings from a consanguineous family possibly associated with maternal hyperhomocysteinemia

Conotruncal defects represent an anatomically heterogeneous group of cardiac malformations affecting the outflow tract of the ventricles and the arterial pole of the heart. The exact etiology of congenital heart diseases is unknown. A 31-year-old woman who had three offspring with complex conotruncal cardiac anomalies after consanguineous marriage was reported. The first child is still alive. However, the second affected child died at the age of 3 years. Fluorescence in situ hybridization studies of the siblings excluded CATCH(22) chromosomal abnormality. The maternal laboratory work-up was unremarkable except for low serum folic acid and cobalamin levels and high homocysteine levels. The woman received high dose pyridoxine, cobalamin and folate treatment preconceptionally, and she became pregnant. She delivered a healthy male infant without any abnormalities. Six months later, she became pregnant again without any preconceptional medications. When she was examined during the fourth pregnancy, unfortunately the fetus was found to have a restrictive ventricular septal defect, a right ventricle with two outflows and a right aortic arch. Her last pregnancy was terminated at 22 weeks. The 22-week-old female fetus was examined postmortem and the diagnosis of congenital heart disease was confirmed. Cobalamin and folate administration may help to reduce the development of cardiac malformations.

Closure of Restrictive Ventricular Septal Defects through a Right Axillary Thoracotomy

To report the midterm results of an alternative minimally invasive approach for closure of ventricular septal defects (VSD) through a muscle-sparing minithoracotomy at the right midaxillary line. From September 2003 to December 2005, 22 patients (median age, 5 years; range, 1.5-12 years) underwent a right lateral axillary thoracotomy for closure of a perimembranous VSD. Special features of the approach included an incision between the anterior and posterior axillary fold, a muscle-sparing preparation over the fourth intercostal space, and the establishment of cardiopulmonary bypass with inguinal and superior vena cava cannulation. The ascending aorta was cross clamped and cardioplegic arrest was instituted. The VSDs were all approached through a right atriotomy and closed directly (17 patients) or with a patch (5 patients). Three patients underwent concomitant aortic valve repair. There was no mortality or significant surgical morbidity. Median cross-clamp and cardiopulmonary bypass times were 46 and 104 min, respectively. Follow-up was complete and ranged from 2 to 22 months (median, 20 months). All patients were in sinus rhythm. Echocardiography revealed no residual defects with competent aortic and tricuspid valves. The length of the incision ranged from 4 to 6 cm, was away from the mammary gland, and remained entirely covered by the adducted arm. All children recovered right shoulder function within days, and cosmetic results were very satisfying. One patient developed iliac artery stenosis following inguinal artery cannulation. Closure of perimembranous VSDs in older children can be safely performed through a right axillary thoracotomy without compromising the accuracy of the repair. The cosmetic results are excellent.

Accessory Tricuspid Valve Tissue in Tetralogy of Fallot Causes Hemodynamic Changes During Intermittent Positive-Pressure Ventilation

c a i fl i s m S m t RESENCE OF ACCESSORY tricuspid valve (ATV) tissue is a rare phenomenon. The association of tetralogy of allot (TOF) with ATV is even rarer but has been described ith a restrictive ventricular septal defect (VSD).1 An ATV has ven been found to be associated with complete obstruction of VSD in a human heart.2 The diagnosis of ATV by 2-dimenional echocardiography may be missed due to greater emphais being placed on the associated major anomaly.3 If missed, n ATV may cause problems during weaning from cardiopulonary bypass (CPB).4 The authors describe a child with TOF resenting with severe hemodynamic changes in the operating oom. The cause of this unusual presentation could not be scertained until the ATV was found perioperatively.

Ventricular Septal Defect and Aortic Valve Regurgitation: Pathophysiology and Indications for Surgery

As the velocity of a fluid increases a low-pressure zone is created, this is the Venturi effect and it explains the pathogenesis of aortic valve prolapse (AVP) and aortic insufficiency (AI) that is observed in a subset of patients with a ventricular septal defect (VSD). The VSDs complicated by AI are restrictive with high velocity shunting through the VSD, creating a low-pressure zone that impacts the adjacent aortic valve cusp resulting in AVP and subsequent AI. AVP and AI are therefore acquired lesions. AI is absent at birth because the forces necessary to create the low-pressure zone within the restrictive VSD do not exist in utero. The risk of development of AI increases during childhood, peaking at 5 to 10 years of age. VSD closure eliminates the low-pressure zone that is the cause of ongoing aortic valve cusp deformity and, if performed early, prevents development of AI. Patients with a subarterial VSD and AVP should undergo surgery to prevent the development of AI because this complicates about half of subarterial VSDs with AVP and spontaneous closure is rare. Patients with perimembranous VSDs with AVP should be followed with serial echocardiography and undergo VSD closure if more than trivial AI develops.

1114-204 Regression of left ventricular dilation in children with restrictive ventricular septal defects with initial large volume overload

1114-204 Regression of left ventricular dilation in children with restrictive ventricular septal defects with initial large volume overload