Congenital Aortic Regurgitation Due to Absent Aortic Cusps and High-Degree Mitral Stenosis

Congenital absence of aortic cusps leads to severe aortic regurgitation. We present a newborn with this rare entity with extreme mitral stenosis. Hemodynamic features were those of hypoplastic left heart syndrome. Surgical management consisted of initial modified Norwood procedure followed by orthotopic heart transplantation.     

Tetralogy of Fallot with Aortic Stenosis: Repair Without Aortic Valve Intervention

A 14-day-old boy with tetralogy of Fallot (ToF), ductus-dependent pulmonary circulation, and significant aortic valve stenosis (AVS) underwent primary repair of ToF without aortic valve intervention. The pressure gradient through the aortic valve decreased spontaneously after the operation. This may have been due to the reduction of blood flow through the aortic valve postoperatively. For patients with ToF, AVS, and ductus-dependent circulation, ToF repair alone may alleviate the severity of the AVS.     

Balloon Valvuloplasty for Congenital Aortic Valve Stenosis in an Infant and Children

Percutaneous balloon aortic valvuloplasty (BAV) was performed in 14 patients, including one critically ill infant with congenital valvular aortic stenosis (AS). BAV was effective in 13 patients (except the infant). The peak systolic pressure gradient between the left ventricle (LV) and the ascending aorta decreased from 76.6 ± 21.6 to 29.5 ± 15.3 mmHg ( P < 0.001). Follow-up cardiac catheterization was performed for eight patients between 1 and 3 years (1.6 ± 1.1 years) after BAV. Restenosis was found in only one patient, and the efficacy of BAV continued significantly. Aortic regurgitation developed or increased in severity in 5 of 13 children immediately after BAV. Any other severe complication was not observed.
Dilatation by BAV was not sufficient for the infant with critical AS, and acute myocardial infarction (AMI) in the lateral wall of the LV occurred during the BAV procedure. The infant died 3 days after the procedure due to AMI.
It was concluded that the retrograde double balloon technique was superior to the retrograde single balloon technique. In two cases, the single balloon technique was ineffective because it was impossible to fix the balloon at the aortic annulus. However, the double balloon technique was effective in every patient.
BAV is effective for AS in children, and an optional repeat trial may enable BAV to be the first choice for AS. Although BAV may be effective for neonates and infants with critical AS as an emergency treatment, much attention must be paid during the procedure.     

Management of Congenital Aortic Stenosis

72 patients with congenital aortic stenosis whose ages ranged from 1 month to 15 years were under supervision for periods ranging from several months to more than 9 years. The obstruction was subvalvar in 3. Additional malformations were aortic regurgitation, persistent ductus arteriosus, coarctation of the aorta, ventricular septal defect, partial anomalous pulmonary venous drainage, and mitral stenosis. Bacterial endocarditis complicated 3. Of 5 deaths unrelated to operation, 3 were sudden. Surgical treatment was carried out in 12 patients (commissurotomy, valve replacement with or without previous commissurotomy, subvalvar resection) with 2 deaths. 52 patients remained symptom-free. Detailed investigation to assess the patient for surgical treatment is indicated if symptoms are present or electrocardiographic evidence of severe left ventricular hypertrophy appears. Commissurotomy is, where possible, deferred until an age at which valve replacement can be undertaken if the stenosis cannot be adequately relieved without causing substantial regurgitation.     

Autologous Ross Operation for Congenital Aortic Stenosis

Congenital aortic stenosis is a relatively common cardiac anomaly encountered in approximately 5% of all children with heart disease. The Ross procedure is increasingly used for replacement of the aortic valve in children. We report a 12-year-old boy who was born with congenital aortic stenosis secondary to a bicommissural aortic valve. The patient underwent open valvotomy in infancy and aortic valvuloplasty 2 years later. Residual/recurrent stenosis prompted referral for aortic valve replacement, and he underwent an autologous Ross procedure, in which the aortic root was replaced with a pulmonary autograft and the repaired aortic valve was used to restore right ventricular-to-pulmonary artery continuity. The postoperative course was unremarkable. Nitroprusside, esmolol, and labetolol were used to control postoperative hypertension. He was discharged 4 days after surgery on oral furosemide and aspirin, and he has had no cardiovascular symptoms during follow-up. Recent echocardiography demonstrated mild right ventricular outflow tract obstruction with a peak velocity of 3.6 m/sec, with a gradient of 42 mmHg and moderate pulmonary insufficiency. There was no left ventricular outlet tract obstruction or aortic insufficiency.     

Aortic Regurgitation Associated With Critical Aortic Stenosis in a Fetus

Aortic regurgitation in association with aortic stenosis is rare in the fetus. Findings have shown that severe aortic regurgitation is worsened by the increase in systemic vascular resistance after birth, resulting in low cardiac output, hypoxemia, and neonatal death. This report describes a unique case of aortic regurgitation with aortic stenosis, severe mitral regurgitation, retrograde flow in the aortic arch, and an enormous left atrium with a restrictive foramen ovale in a fetus. In this case, aortic regurgitation was diminished immediately after birth, indicating that spontaneous improvement in aortic regurgitation after birth should be taken into account when the final prognosis is predicted.     

Management Strategy for Very Mild Aortic Valve Stenosis

It is unclear how often patients with very mild aortic stenosis (gradients <25 mmHg) need interval follow-up. The purpose of this study was to define the determinants of disease severity progression and to propose appropriate management strategies. It is known that congenital aortic stenosis is a progressive disease that requires long-term follow-up at consistent intervals. We studied 89 patients with very mild aortic stenosis. Cox proportional hazard modeling was performed to ascertain predictors of morbidity and mortality. Events were defined as valve surgery or death. Of the original 89 patients, 7 died (92% survival); one death was sudden and unexplained and six were noncardiac. Eighteen individuals were lost to follow-up (10 not located and 8 refused participation). Twelve (17%) had valve surgery. The minimum time interval between initial diagnosis of very mild aortic stenosis and surgery was 4.6 years (mean, 14.0). Age at diagnosis, gender, initial gradient, initial gradient/age, and aortic regurgitation were found not to be predictive of outcome. However, the slope of the transaortic gradient [change of gradient/time (years)] was predictive of outcome (hazard ratio of 1.69; confidence interval, 1.4–2.2). At least 17% of these patients progress to require operation. For patients with a gradient slope <1.1, evaluation every 4 or 5 years is recommended. For patients with a gradient slope >1.2, evaluation every 1 or 2 years seems prudent.     

Echocardiographic Follow-Up of Congenital Aortic Valvular Stenosis

We investigated the morphology of the stenotic aortic valve, the progression of the stenosis, and the onset and progression of aortic regurgitation (AR) in patients with congenital aortic valvular stenosis (AVS). The medical records of 278 patients with AVS were reviewed, with the patients with concomitant lesions besides AR excluded. Very mild aortic stenosis was defined as a transvalvular Doppler peak systolic instantaneous gradient (PSIG) less than 25 mmHg, mild stenosis as 25–49 mmHg, moderate stenosis as 50–75 mmHg, and severe stenosis as more than 75 mmHg. The mean age of the patients was 4.9 ± 4.3 years (range, 3 days to 15 years), and 203 (73%) were male. The number of the cusps was determined with two-dimensional echocardiography in 266 patients (95%): unicuspid in 3 patients (1%), bicuspid in 127 patients (48%), and tricuspid in 136 patients (51%). A total of 192 of all patients were followed for 2 months to 14.6 years (mean 4.2 ± 3.3 years) with medical treatment alone. Among 72 patients with very mild stenosis at initial echocardiographic examination, 20% had mild, 3% moderate, and 1% severe stenosis after a mean period of 3.7 years. In 70 patients with mild stenosis at initial echocardiographic examination, 28% had moderate and 9% severe stenosis after a mean period of 5 years. Among 44 patients with moderate stenosis at initial echocardiographic examination, 36% had severe stenosis after a mean period of 3.7 years. Among 192 patients, 40% had AR (3% trivial, 28% mild, and 9% moderate) at initial echocardiographic examination. After a mean period of 4.2 years, 58% of the patients had AR (13 % trivial, 25% mild, 16% moderate, and 4% severe). There was not statistically significant difference between catheterization peak systolic gradients (47 ± 16 mmHg) and Doppler estimated mean gradients (45 ± 9 mmHg) (p = 0.53), whereas Doppler PSIGs (74.9 ± 15.7 mmHg) were higher than catheterization peak systolic gradients (p < 0.0001) in 25 patients who were studied in the catheterization lab. Patients with very mild stenosis may be followed with a noninvasive approach every 1 or 2 years, and an annual follow-up is suggested for patients with mild stenosis. Nearly one-third of patients with moderate stenosis at initial echocardiographic examination had severe stenosis after a mean period of 3.7 years. Therefore, we recommend, that patients with moderate stenosis undergo noninvasive evaluation every 6 months. Doppler estimated mean gradient is very useful in predicting the need for intervention in children with AVS.     

Pressure Recovery in Pediatric Aortic Valve Stenosis

This study was designed to evaluate the phenomenon of pressure recovery in pediatric patients with aortic stenosis and also to evaluate how observed differences between catheter and Doppler gradients can be predicted by Doppler echocardiography. Doppler measurements of aortic valve stenosis gradients are known to overestimate observed gradients in the catheterization laboratory. Pressure recovery has been shown to be a contributing factor to this discrepancy. However, the clinical relevance of correcting Doppler gradients using the pressure recovery equation has not been evaluated in the pediatric population. Simultaneously obtained catheter and Doppler gradients were studied in 14 patients (range, 0.03–18 years; mean, 4.1 years) with aortic valve stenosis. A total of 23 data points were measured because 9 patients underwent balloon valvuloplasty and had both a pre- and a post-balloon valvuloplasty data point in the study. The catheter gradients were then compared to peak, mean, and pressure recovery corrected Doppler gradients. Pressure recovery was calculated using a previously validated equation. As expected, measured echocardiographic continuous-wave peak Doppler gradients overestimated the observed catheter gradients (range, 16–93 mmHg; mean, 43 mmHg). The continuous-wave peak Doppler gradients, mean, and pressure recovery adjusted gradients were equally as good in correlating the observed catheter gradients to those obtained by Doppler echocardiography (r = 0.92). However, pressure recovery corrected Doppler gradients were in better agreement with catheter gradients than echocardiographic mean or peak Doppler gradients (95% limit of agreement: –9 to 19 mmHg for pressure recovery corrected gradients, –30 to 11 mmHg for mean Doppler gradients, and 2–83 mmHg for peak Doppler gradients). Measured continuous-wave peak Doppler gradients consistently overestimated catheter gradients. The noted differences may be predicted using the pressure recovery equation. Pressure recovery is a significant factor in children with aortic valve stenosis.     

Normalization of Pulmonary Vascular Resistance Long After Successful Primary Sutureless Repair for Congenital Pulmonary Vein Stenosis

A 3-month-old infant with perimembranous ventricular septal defect (VSD) and pulmonary hypertension presented at our center. Cardiac catheterization showed a systolic pulmonary-to-systemic arterial pressure ratio of 0.99 and a pulmonary vascular resistance of 9.32 μm². Pulmonary angiography showed focal orifice stenosis in the right lower and left lower and upper pulmonary veins, whereas the right upper pulmonary vein was atretic. Sutureless pericardial marsupialization concomitant with VSD patch closure was used to repair the three stenotic veins. Administration of home oxygen therapy and sildenafil citrate was continued after surgical repair. Postoperative catheterization 1.5 years after surgery showed patency of the three repaired veins and normalization of pulmonary vascular resistance.     

Repair of Congenital Ascending Aortic Aneurysm

We report a case of congenital ascending aortic aneurysm associated with double-outlet right ventricle that has been successfully repaired by graft replacement and extracardiac total cavo-pulmonary connection.     

Predictors of Left Ventricular Performance After Valve Replacement in Children and Adolescents with Chronic Aortic Regurgitation

Aortic valve replacement has been recommended in patients who have severe symptoms, in patients with extreme left ventricle (LV) dilatation (end diastolic dimension >4 SD above normal) or LV ejection fraction <50%. However, the occurrence of advanced symptoms or severe LV dilatation raises concern about irreversible LV dysfunction. This study sought to determine the influence of preoperative symptoms, LV size and function on mortality, and postoperative LV performance in children and adolescence after valve replacement for aortic regurgitation (AR). A total of 49 patients 18 years old or younger (mean, 13.9 ± 3) who underwent valve replacement for chronic AR between 1991 and 2001 were followed up for 1–10 years (mean, 3.3 ± 2.1). Baseline and postoperative characteristics were compared between 13 patients (group 1) with extreme LV dilatation and 34 patients (group 2) with a lesser degree of LV enlargement. Preoperative low ejection fraction (p < 0.008), extreme LV dilatation (p < 0.05), and LV end systolic dimension >4 SD above normal (p < 0.05) were predictors of persistent LV dysfunction. Extreme LV dilatation (p < 0.0003), LV end systolic dimension (p < 0.0007), and reduced LV ejection fraction (p < 0.01) predicted persistent LV dilatation. In the setting of chronic AR, preoperative symptoms, LV systolic function, and LV internal dimensions are the main predictors for persistent LV dysfunction and dilatation. Surgical correction should be performed before LV systolic dysfunction and/or extreme LV enlargement occurs.     

Noninvasive Estimation of Aortic Valve Areas in Children with Aortic Stenosis

ABSTRACT. Fifteen children with aortic stenosis were examined with Echo-Doppler cardiography in order to evaluate the use of the continuity equation to calculate the aortic valve area. The results were compared to invasive estimates of valve area calculated with the Gorlin's equation. A close correlation between the two methods, r=0.94, was found and it is concluded that even in children an accurate estimate of valve area can be expected with the use of the continuity equation.     

Aortic Valve Prolapse and Aortic Regurgitation in Patients with Ventricular Septal Defect

Aortic valve prolapse (AVP) was detected in 82 (7.5%) of 1096 patients with ventricular septal defect (VSD) (in 50 at initial echocardiographic examination and in 32 at follow-up) by echocardiography. Of 82 patients with AVP, aortic regurgitation (AR) was detected in 53 (65%) (in 28 at initial echocardiographic examination and in 25 at follow-up), resulting in an incidence of AR of 4.8% (of VSD). The percentage of AVP (20.8%) and AR (16.7%) in muscular outlet VSDs was larger than the percentage of AVP (10.6%) and AR (6.8%) in perimembranous VSDs (p <0.05). Fourty-four patients were followed medically after AVP appeared (3 months to 10.8 years; median, 2.1 years). Initially, there was no AR in 24 of these patients, trivial AR in 7, and mild AR in 13. Trivial AR developed in 6 (25%) and mild AR developed in 3 (13%) of 24 patients who had no AR (in 5 of them within 1 year and in 9 of them within 2 years). In 2 (29%) of 7 patients, trivial AR progressed to mild AR during a median of 2 years, and in 4 (31%) of 13 patients, mild AR progressed to moderate AR during a median of 1.1 years. We recommend frequent echocardiographic evaluation (every 6 months) for detecting of appearance of AR in patients with perimembranous or muscular outlet VSD after AVP develops and for evaluating the progression of AR in patients with perimembranous or muscular outlet VSD, AVP, and trivial AR. In addition, we recommend surgical intervention in patients with perimembranous or muscular outlet VSD, AVP, and mild AR because of rapid progression of mild AR to moderate AR.     

Pulmonary Stenosis is a Predictor of Unfavorable Outcome After Surgery for Supravalvular Aortic Stenosis

We sought to evaluate whether the presence of pulmonary stenosis (PS), amongst other factors, influences the mortality and the rate of reoperations in the long-term follow-up of patients with supravalvular aortic stenosis (SVAS). We identified all patients with SVAS from our surgical database. The patients with multi-level aortic stenosis or concomitant cardiac procedures were excluded from this study. Follow-up (100?%) was conducted between 2008 and 2010. Twenty-six patients underwent surgery for SVAS between 1974 and 2006. Seventeen patients (65?%) were diagnosed with Williams-Beuren-Syndrome, six (17?%) had a diffuse form of SVAS and 10 (39?%) had PS. No patient had a surgical or interventional procedure for PS at the initial operation or during follow-up. There was no statistically significant association between PS and WBS (p?=?0.30) or diffuse form of SVAS (p?=?0.13). Patients with PS were operated at younger age (p?=?0.028). Median follow-up time was 14.6?years. Overall mortality was 11.5?%. One patient with preoperatively severely decreased LV-function died 27?days postoperatively. Two late deaths occurred 7 and 10?years after the initial operation. Reoperations were required in 4 patients (15?%), 4-19?years after the original operation, due to aortic arch stenosis, supravalvular restenosis or poststenotic aortic dilatation. PS was found to be a risk factor for reoperation (p?=?0.005) and for the combined reoperation/death end-point (p?=?0.003). PS in patients with SVAS is a risk factor for reoperations in the aortic region and might be considered an indicator of the severity of the arterial disease and a predictor of an unfavourable outcome.