Unrestrictive Ventricular Septal Defect Research Articles

Four-chamber view of the patient with color flow for the unrestrictive ventricular septal defect.

Four-chamber view of the patient with color flow for the unrestrictive ventricular septal defect.

Four-chamber view featuring the unrestrictive ventricular septal defect.

Four-chamber view featuring the unrestrictive ventricular septal defect.

Pseudoaneurysm complicating right ventricle–to–pulmonary artery conduit surgery: Incidence and risk factors

ObjectivesAlthough pseudoaneurysm is an uncommon complication after right ventricle–to–pulmonary artery conduit placement, it has the potential to cause significant morbidity and mortality. MethodsWe performed a review of patients with pseudoaneurysms diagnosed at our institution in a 20-year period (from 1995 through 2015) and compared their clinical characteristics with a group of age- and sex-matched control patients. ResultsWe found that younger age, smaller size, the diagnosis of tetralogy of Fallot, the use of a pulmonary homograft conduit, the presence of an unrestrictive ventricular septal defect after conduit placement, and having at least systemic right ventricular pressure were all more common in patients who had pseudoaneurysms develop. ConclusionsThis study is unique in identifying both patient and surgical factors that may predispose to pseudoaneurysm development and can help inform optimal strategies to monitor and evaluate this patient population.

Left Ventricular Retraining and Double Switch in Patients With Congenitally Corrected Transposition of the Great Arteries

Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart defect with numerous anatomic subgroups. The majority of patients with CC-TGA are excellent candidates for a double-switch procedure. However, in the absence of an unrestrictive ventricular septal defect or subpulmonary stenosis, the left ventricle (LV) may undergo involution and require retraining prior to double switch. The purpose of this study was to review our experience with patients having CC-TGA who required LV retraining prior to a double-switch procedure. This was a retrospective review of 24 patients with CC-TGA who were enrolled in an LV retraining program in preparation for a double-switch procedure. The median age at the time of enrollment for retraining was 11 months (range 1 month-24 years). The average left ventricle to right ventricle pressure ratio was 0.39 ± 0.07 prior to intervention. All 24 patients underwent placement of an initial pulmonary artery band (PAB) for LV retraining. Eighteen (75%) of the 24 patients underwent a double-switch procedure with no operative mortality. Of these 18 patients, 9 had a single PAB and 9 required a second band for retraining. Six patients have not undergone a double-switch procedure to date. Five patients are good candidates for a double switch and are 2 weeks, 3 weeks, 4 weeks, 8 months, and 35 months since their last PAB. One patient died from a noncardiac cause 26 months after PAB retightening. The 18 patients who underwent a double switch were followed for an average of 5 ± 1 years (range 0.1-10.3 years). There has been no late mortality, and only 2 patients required further reinterventions. The data demonstrate that LV retraining has been highly effective in this select group of patients with CC-TGA. The data also demonstrate that the results of the double-switch procedure have been excellent at midterm follow-up. These results suggest that LV retraining and double switch offer a reliable strategy option for patients with CC-TGA.

Palliative balloon pulmonary valvuloplasty for infants with unrestrictive ventricular septal defect or single ventricle associated with severe pulmonary stenosis.

Symptomatic infants with unrestrictive ventricular septal defect (VSD) or single ventricle with severe pulmonary stenosis (PS) are typically treated surgically. High surgical risk infants may benefit from catheter-based palliative pulmonary valvuloplasty. We retrospectively reviewed all patients with severe PS associated with unrestrictive VSD or single ventricle who underwent palliative pulmonary valvuloplasty as initial management and describe outcomes. From 2000 to 2013, 16 patients met inclusion criteria and underwent valvuloplasty at a median age and weight of 25 (range 1-352) days and 3.65 (range 2.2-12) kg. Common diagnoses included tetralogy of Fallot (50%) and double-outlet right ventricle (25%). In all, the indication was cyanosis. Reasons to defer surgery included low weight (37.5%), preference for complex operation at older age (31.25%) and significant comorbidity (18.75%). Following valvuloplasty, significant increases in oxygen saturation (83.1±8.8 vs. 74.7±8.7%, P=0.008) and QP:QS (1.6±0.5 vs. 0.8±0.3, P=0.0005) occurred. All two ventricle patients (n=10) ultimately had complete operative repair at a median of 125 (range 27-382) days after valvuloplasty, during which time there was 20.3±9.2 g/day of weight gain without change in oxygen saturation. Single ventricle patients who underwent cavopulmonary connection (n=3) had similar weight gain and palliation time, but with a decrease in oxygen saturation. Two patients required reintervention to augment QP prior to definitive repair. In select infants with unrestrictive VSD or single ventricle associated with severe PS, palliative pulmonary valvuloplasty provides an effective and durable method of increasing oxygen saturation until definitive surgical treatment can be performed.

Long-term survival with good functional capacity in unoperated complex congenital heart defect with balanced systemic and pulmonary circulation

A 46-year-old woman was admitted to our Department of Cardiology for stable exertional symptoms of heart failure (NYHA I) and distal mild cyanosis. The medical history revealed a complex congenital heart defect (double-inlet ventricle; DIV) diagnosed early at the infancy, but without palliative surgery or radical repair. The patient was leading a near normal life doing all the house work and working as a full-time physical worker. She had three healthy children delivered through a C-section without cardiovascular complications or significant symptoms progression. None of them was diagnosed with a heart defect. Physical examination revealed a blood pressure of 110/70 mmHg, heart rate 70 bpm, distal mild cyanosis (oxygen saturation 71 %), clubbed fingers, loud systolic heart murmur in the precordial area with a left parasternal thrill and only a mild right heart failure symptoms. Electrocardiogram showed a normal sinus rhythm without atrioventricular conduction disturbances. The major routine laboratory tests abnormalities were: Hb-19.85 g/dl, Ht-61.30 %, Fe-50 mg/dl and NT-proBNP-428 pg/ml. Moreover, a few months earlier she was diagnosed a hypothyroidism and a levothyroxine supplementation was introduced. The performed 6 min walk test was used to assess functional capacity showed a maximal distance of 440 m with a desaturation (75–51 %). Transthoracic echocardiography and cardiac magnetic resonance (CMR) imaging showed a double-inlet left ventricle of normal systolic function with unrestrictive ventricular septal defect and rudimentary right ventricle, d-transposition of great arteries. Both atrio-ventricular valves reveal only a mild regurgitation. There are also concordant atria with normal venous return separated with a residual interatrial septum and no pathology within an aortic arch. The most important for a long survival and balanced systemic and pulmonary circulation is that there is normal flow through the aortic valve without any obstruction and a moderate-to-severe pulmonary stenosis with supravalvular pulmonary stenosis, either (Fig. 1). The usual surgical approach in infants is a palliative shunt operation followed by one of the Fontan operation modifications. DIV is believed to carry a poor prognosis if unoperated with most patients dying before 16 y.o. [1]. Survival into adulthood without any surgical treatment is extremely rare and is found only in patients with a left ventricle-type of single ventricle and similar to the presented case combination of heart defects with normal systolic ventricle function and a moderate-to-severe pulmonary stenosis, which seems a crucial condition of balanced pulmonary circulation. The haemodynamic changes during pregnancy may usually significantly worsen cardiovascular symptoms even long time after a delivery. However, our patient denied significant progression of symptoms and there were no complications during or afterwards which suggest a perfectly balanced circulation. CMR provides most necessary information in the complex congenital heart defect, but a heart catheterization may add unique data on pulmonary artery pressure and pulmonary circulation. However the good functional capacity and only a mild cardiovascular symptoms in our patient encourage us to a strict follow-up for ventricular function, arrhythmia and functional capacity. Fig. 1 Cardiac magnetic resonance (a, b) and transthoracic echocardiography (c) showing a left-type single ventricle (LV), an unrestrictive interventricular septum defect with a rudimentary right ventricle (RV), transposition of great arteries (Ao aorta; PA ...

Prenatally Diagnosed Coarctation: A More Sinister Disease?

Prenatal diagnosis of coarctation may improve survival, but little is known regarding its association with severity of left heart hypoplasia and surgical outcome. This study compared echocardiographic measurements of left heart structures, surgical strategies, and postoperative outcomes between neonates with a prenatal diagnosis of coarctation and those with a postnatal diagnosis. All the neonates who underwent coarctation repair during 2006-2010 were reviewed. The neonates with complex congenital heart disease or an unrestrictive ventricular septal defect requiring cardiopulmonary bypass (CPB) for closure were excluded from the study. Based on the time of diagnosis, the subjects were divided into the following three groups: group 1 (prenatal diagnosis), group 2 (diagnosis at 0-7 days), and group 3 (diagnosis at 8-28 days). The study population consisted of 46 neonates: 14 in group 1, 14 in group 2, and 18 in group 3. Compared with group 3, group 1 had a smaller left ventricular volume index, lower mitral and aortic valve diameter z-scores, and lower transverse arch z-scores. Compared with group 2, group 1 had smaller aortic valve z-scores but otherwise had similar measurements. At repair, 64 % of the neonates in group 1 required CPB versus 29 % in group 2 (p = 0.12) and 22 % in group 3 (p = 0.03). All the neonates underwent biventricular repair, with no surgical mortality. Group 1 had a hospital stay of 13.4 ± 10.8 versus 7.5 ± 4.2 days in group 2 (p = 0.06) and 7.3 ± 4.5 days in group 3 (p = 0.03). The neonates with prenatally diagnosed coarctation had smaller left heart structures than the neonates with coarctation diagnosed after the first week of age, were more likely to require extensive arch reconstruction under CPB, and had longer hospital stays.

Late percutaneous re-canalization of arterial duct-dependent isolated pulmonary artery

A critical infant with unrestrictive ventricular septal defect and absence of the left pulmonary artery underwent transcatheter re-canalization of the occluded arterial duct-left pulmonary artery complex and surgical banding of the right pulmonary artery. After this hybrid approach, the baby was discharged under mild anticongestive therapy in view of a later surgical repair.

Hybrid palliation in complex congenital heart malformation with duct-dependent isolated pulmonary artery

A 2 month-old infant with severe congestive heart failure due to unrestrictive ventricular septal defect and absence of the left pulmonary artery was submitted to a hybrid transcatheter-surgical palliation consisting in percutaneous re-canalization of the occluded arterial duct-left pulmonary artery complex and surgical banding of the right pulmonary artery. The post-operative course was uneventful and the baby was discharged in a few days under mild anti-congestive therapy. This hybrid approach was highly effective in stabilizing this critical infant in view of a later and safer surgical repair.

Cardiac Magnetic Resonance Imaging of a Coronary Sinus Diverticulum Associated With Congenital Heart Disease

A 6-month-old girl was referred for surgical repair of tetralogy of Fallot. A transthoracic echocardiogram revealed a large, unrestrictive ventricular septal defect, mild aortic override, a normal pulmonary valve with mild infundibular obstruction (peak gradient 22 mm Hg), a small patent ductus arteriosus, secundum atrial septal defect, and persistence of the left superior vena cava to the coronary sinus. The preoperative ECG showed sinus rhythm, mean frontal plane axis of 130°, right ventricular hypertrophy, and no signs of preexcitation (Figure 1). A chest x-ray showed cardiomegaly and prominent pulmonary vascularity with mild hyperinflation (Figure 2). Under cardiopulmonary bypass, the patient underwent repair consisting of resection of multiple infundibular muscle bundles, patch closure of the ventricular septal defect, suture closure of atrial septal defect, and ligation of the patent ductus arteriosus. She had an uncomplicated recovery, but a routine postoperative echocardiogram before discharge showed an echolucent mass measuring 16×22 mm, posterior and inferior to the …

An unusual case of tricuspid lesion in congenital corrected transposition of the great arteries

An unusual case of severe supravalvular stenosing ring of the left atrium associated with tricuspid valve dysplasia in an adult symptomatic patient affected by congenitally corrected transposition and unrestrictive ventricular septal defect is reported. The stenosis of the systemic atrioventricular valve possibly prevented the development of obstructive pulmonary vascular disease; removal of the membrane, attached to the tricuspid annulus, together with pulmonary banding was carried out. Clinical conditions improved after surgery. The unusual stenotic lesion of the systemic right ventricular inflow allowed conservative surgical palliation in this adult patient.

Effect of cardiopulmonary bypass on urea cycle intermediates and nitric oxide levels after congenital heart surgery

Objective To test the hypothesis that cardiopulmonary bypass used for repair of ventricular septal defects and atrioventricular septal defects would decrease availability of urea cycle intermediates including arginine and subsequent nitric oxide availability. Study design Consecutive infants (n = 26) undergoing cardiopulmonary bypass for repair of an unrestrictive ventricular septal defect or atrioventricular septal defect were studied. Blood samples were collected immediately before surgery, immediately after surgery, and 12 hours, 24 hours, and 48 hours after surgery. Urea cycle intermediates, including citrulline, arginine, and ornithine, were measured by amino acid analysis. Nitric oxide metabolites were measured by means of the modified Griess reaction. Results Cardiopulmonary bypass caused a significant decrease in the urea cycle intermediates arginine, citrulline, and ornithine at all postoperative time points compared with preoperative levels. The ratio of ornithine to citrulline, a marker of urea cycle function, was elevated at all postoperative time points compared with preoperative values, indicating decreased urea cycle function. Nitric oxide metabolites were significantly decreased at all postoperative time points except for 48 hours, compared with preoperative levels. Conclusions Cardiopulmonary bypass significantly decreases availability of arginine, citrulline, and nitric oxide metabolites in the postoperative period. Decreased availability of nitric oxide precursors may contribute to the increased risk of postoperative pulmonary hypertension. (J Pediatr 2003;142:26-30)

Left Aortic Arch and Right Descending Aorta -Interruption or Severe Coarctation of the Aortic Arch in the Newborn

Three consecutive neonates (aged 7, 14 and 30 days, body-weight 2980 g, 3000 g and 3400 g respectively) with interruption of the aorta ( n = 2) and severe coarctation ( n = 1) in the presence of left aortic arch and right descending aorta are reported. Associated lesions were an aortopulmonary window in the first case and an unrestrictive ventricular septal defect in the two others. Intractable heart failure and the complexity of the malformation led to the decision of a staged operation. A prosthetic graft was interposed between the ascending and descending aorta via a right thoracotomy in order to bridge the atretic or hypoplastic segment without using extracorporeal circulation. There was no intraoperative complication. One patient developed a thrombocytopenia within the frame of a sepsis syndrome and died on day 5 after operation, death being caused by a massive bleeding into the left thoracic cavity, although the operation was carried out via a right thoracotomy. Angiography 1 year after operation revealed a good flow through the grafts and no stenosis at the site of the anastomoses. The two surviving infants are clinically well without any medication. The reported operative technique provides an alternative palliative possibility to manage critically ill neonates without any obstacle to later definitive repair.

Left aortic arch and right descending aorta — interruption or severe coarctation of the aortic arch in the newborn

Three consecutive neonates (aged 7, 14 and 30 days, body-weight 2980g, 3000g and 3400g respectively) with interruption of the aorta ( n = 2) and severe coarctation ( n = 1) in the presence of left aortic arch and right descending aorta are reported. Associated lesions were an aortopulmonary window in the first case and an unrestrictive ventricular septal defect in the two others. Intractable heart failure and the complexity of the malformation led to the decision of a staged operation. A prosthetic graft was interposed between the ascending and descending aorta via a right thoracotomy in order to bridge the atretic or hypoplastic segment without using extracorporeal circulation. There was no intraoperative complication. One patient developed a thrombocytopenia within the frame of a sepsis syndrome and died on day 5 after operation, death being caused by a massive bleeding into the left thoracic cavity, although the operation was carried out via a right thoracotomy. Angiography 1 year after operation revealed a good flow through the grafts and no stenosis at the site of the anastomoses. The two surviving infants are clinically well without any medication. The reported operative technique provides an alternative palliative possibility to manage critically ill neonates without any obstacle to later definitive repair.

1002-88 Is Angiocardiography Necessary in the Preoperative Assessment of Uncomplicated Ventricular Septal Defect in Infancy?

With current 2-dimensional echocardiography (ECHO) and colour-flow Doppler it may no longer be necessary to perform preoperative angiocardiography (ANGlO) for patients with a primary diagnosis of uncomplicated unrestrictive ventricular septal defect (VSD). We compared the sensitivity and specificity of both techniques in the detection of additional VSD's and commonly associated lesions using as “gold standard” a combination of surgical findings plus postoperative ECHO's. A total of 130 consecutive patients undergoing VSD repair in infancy between January 1991 and March 1994 were studied. The mean age at repair was 0.48 ± 0.26 year and mean weight at repair was 5.0 ± 1.5 kg. All ECHO's were performed prior to ANGlO. Associated lesions included right ventricular muscle bundles (RVMB), sub-aortic stenosis (sub-AS), and persistent ductus arteriosus (PDA). No case of aortic valve prolapse causing aortic incompetence was identified, and aortic arch abnormalities were generally surgically addressed prior to ANGlO. The sensitivity and specificity of the two techniques in the detection of additional VSD's were: Sensitivity Specificity Echo 63% 99% Angio 63% 95% Out of a total of 8 cases with additional VSD's, 2 were small anterior muscular defects which were clinically insignificant, and only seen by colour-flow Doppler. The results for detection of associated lesions were: Sensitivity Specificity Echo Angio Echo Angio RVMB 73% 73% 93% 96% Sub-AS 63% 38% 96% 97% PDA 80% 80% 98% 99% Our results suggest that ECHO is comparable to ANGlO in the preoperative assessment of uncomplicated VSD in infancy. Therefore, ANGlO should no longer be necessary in the evaluation of these patients.

Pulmonary arterial development in infants with large perimembranous ventricular septal defects associated with overriding of the aortic valve

Intracardiac anatomy and pulmonary vascular structure were studied in 13 cases having large perimembranous ventricular septal defects associated with overriding of the aortic valve, 12 of whom died aged 2 days–9 months. In all cases the ventricular septal defect and aorta were of the same size, but the diameter of the pulmonary orifice was usually greater. Quantitative morphometric techniques were used to study the lungs. Structural adaptation to extra-uterine life was shown to have occurred normally in the 4 patients who died as neonates. In the cases aged 2 months–4 years, there was an increase in arterial medial thickness and extension of muscle, while the intra-acinar arteries were larger than normal in size and normal in number. Intimal proliferation was seen in 1 case at 4.5 months, but such proliferation caused significant obstruction only in one child aged 4 years. These findings emphasize the rapidity with which structural adaptation to extra-uterine life can occur in the patient with a large unrestrictive ventricular septal defect. A rapid increase in muscularity can be seen by 2 months of age. Should banding of the pulmonary artery be required in patients with more complicated intracardiac abnormalities in whom it is desirable to achieve a normal pulmonary vascular bed, our findings encourage its early performance, particularly in those destined for a Fontan repair.