Severe subvalvular pulmonic stenosis due to a ball-shaped accessory tissue in an asymptomatic woman with congenitally corrected transposition of great arteries and mesocardia: A rare case report.

Congenitally corrected transposition of great arteries (CCTGA) is a rare anomaly described by atrioventricular and ventriculoarterial discordance. On the other hand, mesocardia that is extremely rare includes two relatively well-defined apexes defined by each ventricle with the major axis of the heart lies in the midline. We describe a rare case of an asymptomatic 20-year-old woman with mesocardia, CCTGA, and severe subvalvular pulmonic stenosis due to a ball-shaped accessory tissue in left ventricular outflow tract.

Transcatheter right ventricular outflow tract stenting in children with postoperative infundibular stenosis and preserved pulmonary valve function.

Recurrent or residual right ventricular outflow tract obstruction after early surgical repair of congenital heart disease is one of the most frequent indications for either surgical or transcatheter reintervention. Transcatheter stent implantation across the stenotic right ventricular outflow tract or conduit is a safe and effective alternative to surgical reintervention. However, chronic deleterious effects of pulmonary regurgitation can potentially counterbalance the early improvement in clinical and hemodynamic parameters, sometimes necessitating further intervention. While there are several studies documenting safe and effective palliation by transcatheter right ventricular outflow tract stenting in infants with tetralogy of Fallot, literature on isolated infundibular stent implantation sparing the normal pulmonary valve in postoperative infundibular restenosis is very scant. We report our experience of safety and feasibility of transcatheter right ventricular outflow tract stent implantation while preserving the native pulmonary valve function in two children with infundibular stenosis after surgical repair of congenital heart disease.

Development of supravalvular pulmonary artery stenosis following a Nuss procedure.

We report a case of a 13-year old girl with pectus excavatum who had a Nuss procedure and two years later a new cardiac murmur appeared which on investigation was diagnosed as supravalvular pulmonary artery stenosis. Following removal of the Nuss bar the stenosis resolved.

LEOPARD syndrome.

LEOPARD syndrome (LS) is a rare hereditary disorder, characterised mainly by skin, facial and cardiac abnormalities. We report on the case of a six-year-old Djiboutian with typical features of LS. Multiple cardiovascular problems are described, including pulmonary infundibular, valvular and supra-valvular stenosis. A favourable course was observed after successful cardiac surgery. This is the first reported case of LS from the horn of Africa.

Acute effects of DDD pacing in patients with pulmonary infundibular stenosis.

We evaluated acute effects of DDD pacing (right atrium sensed and left ventricle paced) in 3 patients with pulmonary infundibular stenosis and found a decrease in dynamic right ventricular outflow gradient in all of them. It appears that acute temporary DDD pacing may decrease the dynamic obstruction of the right ventricular outflow tract in these patients, probably because of asynchronous contraction of the right ventricle induced by pacing from the left ventricular apex, with contraction of infundibular portion being delayed.

Surgical repair of pulmonary stenosis with intact ventricular septum in a 68-year-old woman.

Patients with mild pulmonary stenosis after infancy rarely have symptoms or develop increasing obstruction. We experienced a 68-year-old woman with severe pulmonary valvar and infundibular stenosis (peak to peak pressure gradient = 80 mmHg). She had been pointed out heart disease at the age of six. Endocarditis at the age of 17 might induce calcification of valve and affect the progression of pulmonary stenosis, and moreover, which might gradually develop severe subvalvar obstruction and poststenotic aneurysm of pulmonary trunk. She refused operative intervention because of mild clinical symptoms (NYHA class II), but we recommended surgical repair due to repeated transient ischemic attacks, which were suspected paradoxical embolism through persistent foramen ovale. She underwent pulmonary valvotomy and infundibular resection and is doing well.

O acometimento isquêmico obstrutivo na cardiomiopatia hipertrófica do tipo septal assimétrico com envolvimento da artéria descendente anterior / Obstrutive ischemic involvement of the left anterior descending coronary artery in asymmetrical septal form of hypertrophic cardiomyopathy

OBJETIVO - Avaliar o papel da doença coronária (DAC) com comprometimento da artéria descendente anterior (ADA) na cardiomiopatia hipertrófica (CMH) e sua repercussão na evolução, visto ser controverso o significado da necrose e fibrose do septo interventricular (SIV) nesta cardiomiopatia. MÉTODOS - Entre 158 pacientes com CMH, selecionados 6 (3,79 'por cento') com CMH e DAC com lesão obrigatória de ADA, sendo 4 homens, entre 52 e 70 (x=65,16) anos, 4 com a forma obstrutiva da CMH. O tempo de diagnóstico da CMH foi de 78 a 182 (x=141) meses e da DAC de 1 dia a 106 (x=42) meses. Os pacientes foram acompanhados com avaliaçöes clínicas e exames complementares periódicos. RESULTADOS - A forma de apresentação da DAC foi em 5 com angina instável e um com infarto do miocárdio. A ADA estava comprometida entre 60 a 100 'por cento', sendo em um lesão única e nos 5 restaurantes com lesão em 2 ou mais vasos. Na evolução, 3 foram submetidos a revascularização miocárdica (RM), um associada a miomectomia septal, um a angioplastia e 2 somente a tratamento clínico. No período de observação de 76 a 124 meses após o diagnóstico da DAC, ocorreu um óbito. No fim do estudo observamos redução nos valores médios do SIV de 1,53 para 1,40cm, gradiente de pressão entre o corpo e a via de saída do ventrículo esquerdo (VE) de 56 para 15,75mmHg, com discreto aumento no diâmetro diastólico do VE de 4,55 para 4,85cm e do diâmetro sistólico de 2,83 para 3,13cm, sem alterar a dimensão do átrio esquerdo (4,13cm). CONCLUSÄO - A DAC da ADA é bem tolerada na CMH septal assimétrica, participando do processo fibrótico septal e melhorando o desempenho cardíaco, não representando problema adverso na evolução da CMH

[Non-surgical ventricular training before arterial switch. Focus on an animal model in lambs]. / Préparattion non chirurgicale du ventricule sous-pulmonaire à la détransposition artérielle. Mise au point d'un modèle animal chez l'agneau.

One of the conditions for successful anatomical correction of transposition of the great arteries (arterial switch) or of double discordance (double switch) is the ability of the subpulmonary left ventricle to adapt to the systemic circulation when restored to its subaortic position. A balloon catheter designed for training the subpulmonary ventricle by progressive occlusion of the main pulmonary artery (NuMed) was implanted in six lambs aged 45 days and inflated progressively to obtain the maximum tolerated right ventricular pressure. Six controls were instrumented without balloon inflation. The experiment lasted 5 days. Progressive inflation of the balloon to a right ventricular pressure > 70% of the systemic (carotid) pressure led to bradycardia with venous desaturation and acidosis which regressed when the balloon was deflated. In one animal, progressive adjustment enabled a right ventricular pressure of 75% of the systemic pressure to be obtained throughout the last day of the study. No significant right ventricular hypertrophy was obtained. The value of this technique is in assessing the afterload reserve of the tested ventricle before surgical banding of the pulmonary artery, the first step to anatomical correction for restoration of concordance of double discordance or of transposition of the great arteries previously treated by a Senning or Mustard procedure.

[Intermediate and long-term outcome after percutaneous balloon dilatation of valvular pulmonary stenoses in childhood]. / Mittel- bis langfristige Ergebnisse nach perkutaner Ballondilatation valvulärer Pulmonalstenosen im Kindesalter.

Pulmonary balloon valvuloplasty was performed in 52 patients aged 7 days to 19 years (mean 5.5 years). Mean balloon/anulus ratio was 1.24. Mean right ventricular outflow tract (RVOT) systolic pressure gradient was 79.9 +/- 37.3 (x +/- SD) mm Hg before valvuloplasty and 37.2 +/- 29.6 mm Hg (p < 0.001) immediately after the procedure. 33 patients had residual RVOT-gradient < or = 36 (22 +/- 7) mm Hg, 19 patients had gradients > 36 (67.1 +/- 35.6) mm Hg. During intermediate follow-up (< 2 years) RVOT gradient assessed by catheterization or Doppler echocardiography improved without any additional intervention in 10/19 patients with RVOT gradient > 36 mm Hg early after valvuloplasty. 6/19 patients required additional reduction of RVOT gradient by repeated valvuloplasty (51 +/- 19 to 29 +/- 5 mm Hg; p < 0.01). 3/19 patients needed surgical resection of extremely thickened dysplastic valves. 49 patients had a mid- to long-term follow-up by echocardiography (mean 4.3, max. 9 years); the RVOT gradient decreased from 25.7 +/- 12.8 mm Hg during intermediate follow-up to 18.0 +/- 7.0 mm Hg during long-term follow-up. If pulmonary regurgitation was present after dilatation (n = 38), it was hemodynamically not significant and did not change during follow-up. Percutaneous balloon valvuloplasty was a safe and effective treatment for pulmonary valve stenosis in infancy and childhood. Long-term results confirm the value of this method.

Usefulness of banding of the pulmonary trunk with single ventricle physiology at risk for subaortic obstruction.

This study addresses the effects of early banding of the pulmonary trunk and subsequent management of subaortic obstruction on the attainment of acceptable pre-Fontan hemodynamics in patients with a single left ventricle and aorta arising from an outflow chamber. We report our experience with 26 patients seen at our institution between January 1984 and December 1994 with a diagnosis of double-inlet left ventricle or tricuspid atresia and transposed great arteries, who were initially managed with pulmonary artery banding in the first 6 months of life. Pulmonary artery band placement was performed at an age of 2.1 +/- 1.8 months (mean +/- SD). Associated aortic arch abnormalities were present in 8 patients (31%). There were 19 patients (73%) who underwent treatment with a Damus-Kaye-Stansel procedure or ventricular septal defect (VSD) enlargement for a significant subaortic gradient or morphologically small VSD, alone or in conjunction with a Glenn or Fontan procedure. Eighteen of 26 patients (69%) underwent cardiac catheterization to assess their candidacy for the Fontan operation. Of this group, 16 were classified as low to moderate risk and 2 as high-risk Fontan candidates, based on hemodynamic criteria. The cumulative mortality for the entire cohort was 19%. Our results suggest that this high-risk group of patients can undergo effective pulmonary artery banding as an initial palliative step, with subsequent intervention for subaortic ob- struction when it is documented or highly suspected, and that acceptable pre-Fontan hemodynamic parameters can be achieved.

Infundibular pulmonic stenosis with intact ventricular septum: a report of 15 surgically corrected patients.

Infundibular pulmonic stenosis with intact ventricular septum of primary origin is an uncommon condition. We report 15 such patients (nine males and six females, aged 7-36 years) who had undergone surgical correction for the anomaly during the period between 1975 and 1992. The occurrence of this clinical setting represents 0.19% (15/7826) of all cardiac operations and 0.46% (15/3222) of congenital heart diseases undergoing surgical correction during that period of time. The lesion was of discrete fibromuscular hypertrophy of the infundibulum in all 15 patients. The presenting symptoms of most patients were exertional dyspnea and syncope; however, five patients with severe obstruction were asymptomatic. The peak systolic pressure gradient across the infundibulum ranged from 71 to 230 mmHg. There was only one operative death; the remainder had remained well following the surgery over a mean follow-up period of 35 months. Surgical correction for infundibular pulmonic stenosis is rewarding in the absence of heart failure.

Early hypercyanotic spells unrelated to ductal constriction in tricuspid atresia with infundibular stenosis.

Clinical, echocardiographic, hemodynamic, and angiographic findings were reviewed in 16 consecutive patients with tricuspid atresia (TA) and normally related great arteries, seen at the University of Florida between 1984 and 1990, to determine when and in which hypercyanotic spells (HS), unrelated to ductal constriction, occurred. Such spells developed in six patients (38%) and were life-threatening and refractory to medical management in three patients. Most HS (five of six) occurred in the first 2 months of life. Angiography done at the time of diagnosis revealed similar morphological features in all patients subsequently experiencing HS; infundibular stenosis due to anterior malalignment in the conal septum. These findings suggest a predisposition for development of early HS in TA patients with apparent infundibular stenosis due to anterior malalignment of the conal septum. Further study and a heightened clinical awareness are needed in such patients.

[Doppler echocardiographic estimates of pressure gradients in various types of stenoses: usefulness and limitations].

UNLABELLED: In the present study, the accuracy of Doppler estimates of pressure gradients in various types of stenoses was clinically and experimentally evaluated. Fifty-seven patients, including 23 with ventricular septal defect, 15 with aortic or pulmonary valvular stenosis, four with infundibular stenosis, and five with supravalvular aortic or pulmonary stenosis were observed. The peak systolic pressure gradient (dP (C] was obtained at the time of catheterization in all patients. Before catheterization, the maximum velocity was measured by pulsed or continuous Doppler echocardiography and the estimated systolic pressure gradient according to Doppler (dP (D] was calculated by the simplified Bernoulli equation. The experimental model was designed to create pulsatile flow through a stenosis model. Nine different stenotic model types were used, including three orifice-like stenoses and six truncated cones with heights of 10 mm and 20 mm distal to the stenosis. The orifices in their stenoses were 3, 4 and 5 mm, respectively. Glycerin solution containing Sephadex with a viscosity similar to that of blood was used as the circulation medium. Its specific gravity was 1.16 g/cm3. In each stenotic model, the maximum velocity and instantaneous systolic peak pressure gradient were measured at various water flow rates. CLINICAL RESULTS: In patients with ventricular septal defect or valvular stenosis, dP (D) correlated very well to dP (C), with the regression equation, y = 0.87x + 2.79 (r = 0.92) or y = 0.96x + 1.02 (r = 0.99). In the other patients except for three with patent ductus arteriosus, dP (D) overestimated dP (C) by 11 to 71 mmHg, and their post-stenotic areas had gradually widened according to angiographic findings.(ABSTRACT TRUNCATED AT 250 WORDS)

Esmolol (Brevibloc) to assess dynamic subpulmonary stenosis in a child after repair of complete transposition: a case report.

Esmolol (Brevibloc), a new, ultra-short acting, cardioselective beta-adrenergic blocking agent with half-life of 9.2 min following i.v. administration was given to a 4-year-old child with known dynamic and fixed sub-pulmonary stenosis post Senning repair for complete transposition of the great vessels. The left ventricular systolic pressure increased from 48 to 100 mmHg, heart rate showed an increase from 65 to 140 bpm, the right femoral arterial pressures decreased from 115/58 to 77/35 mmHg following an infusion of Isoprel. Infusion of esmolol partially relieved the dynamic sub-pulmonary stenosis. There were no adverse effects and esmolol was tolerated well by the child. Esmolol might thus play a role in the pediatric catheterization laboratory during investigational procedures, electrophysiological studies and in the control of rapid supraventricular tachycardia, especially in adolescents with WPW pre-excitation. Esmolol would also be beneficial in emergency treatment of epinephrine or isoproterenol overdosage.