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[Pulmonary stenosis].

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Constriction, Pathologic↗

[Konno procedure for congenital aortic valve stenosis with pulmonary valve stenosis].

Although aortic and pulmonary valve stenosis are among the most common congenital heart defects, the combination of both aortic and pulmonary valve stenosis in the same patient appears to be very uncommon. Accurate diagnosis of combined valvular stenosis is imperative prior to surgical correction, otherwise surgery of one of the lesions may result in an insufficient hemodynamic improvement. A seven-year-old girl with congenital aortic and pulmonary valve stenosis associated with hypoplastic aortic annulus underwent Konno's operation, pulmonary valvotomy and resection of anomalous muscle of the right ventricular outflow tract simultaneously. The operation was successfully performed and postoperative course was uneventful.

Aortic Valve Stenosis↗

Prolonged survival in a patient with a single ventricle without pulmonary stenosis.

Prolonged survival of the patient with a single ventricle is rare. We report the case of a 57-year-old man who has type A3 of Van Praagh's classification of single ventricle with pulmonary hypertension without pulmonary stenosis. Favorable streaming, a large bulboventricular foramen, and intact atrioventricular valves may have contributed to his long-term survival.

Echocardiography, Transesophageal↗

[Cardiac performance before and after Jatene procedure for transposition of the great arteries: with specific reference to post-operative supravalvular pulmonary stenosis].

The cardiac performance was evaluated in patients who had received arterial switch operation (AS-op) for transposition of the great arteries (TGA) in their infancy with special attention to postoperative supravalvular pulmonary stenosis (SVPS). AS-op was performed in 36 infants; 13 with simple TGA and 23 with TGA accompanied with ventricular septal defect. Nine patients had undergone pulmonary arterial banding with systemico-pulmonary shunt. The mean age at surgery was 7 months. Postoperative catheterization was performed on average of 15 months after surgery. The end-diastolic volume (EDV) and ejection fraction (EF) of the left and right ventricles (LV, RV) were calculated from biplane cineangiograms. LVEDV and RVEDV were normalized and expressed as % of normals. SVPS was defined as peak systolic pressure gradient of 50 mmHg or higher between the RV and the pulmonary artery (PA). The mean LVEDV, RVEDV, LVEF and RVEF values were all within the normal range. Inverse correlation was observed between the peak systolic RV-PA pressure gradient and RVEF (n = 29, r = -0.84, p less than 0.001). SVPS after AS-op was observed in 8 patients (22%). Stenosis was observed at the site of anastomosis of the artery. In these cases, the diameter of the PA at the stenotic site decreased to 52% of the preoperative values. Branch stenosis of the PA was observed in 3 patients who had undergone surgery during the neonatal period. Generally, the results after AS-op were satisfactory in terms of cardiac performance. Postoperative SVPS was observed in 8 patients (22%) with decreased RVEF. Careful observations of the growth of PA after AS-op are mandatory.

Cardiac Surgical Procedures↗

Influence of age at time of surgery on pre-operative left ventricular mass and postoperative outcome of Fontan operation in children with tricuspid atresia and native pulmonary stenosis.

Purpose of this study was to examine the influence of early (less than two and half years) versus later (greater than four years) age at time of Fontan type palliation in tricuspid atresia with native pulmonary stenosis on outcome with special reference to left ventricular mass and function. Among the 21 patients with tricuspid atresia, twelve (group A) underwent a Fontan type palliation at a median age of one (.6 to 2.5) years and nine (group B) at a median age of 7.5 (4.8 to 28) years. Left ventricular mass was assessed by cross-sectional echocardiography in the apical two and four chamber view. Mass was calculated as difference between epicardial and endocardial volume x 1.05 (specific gravity of heart muscle). Mass divided by volume at end-diastole yielded the mass/volume index. There was a weak correlation between age and left ventricular mass with an r-value of 0.74. Ejection fraction was calculated from the endocardial volume measurements at end-diastole and end-systole. Patient data were compared to normal values previously established in 95 controls, who were age-matched for the patients. Immediately before surgery left ventricular mass was significantly higher in the nine patients, who underwent surgery at a later age. While postoperative stay in hospital and duration of treatment in the intensive care unit did not differ significantly between both groups, the incidence of pleural and pericardial effusions and the duration of insertion of drainage tubes for these effusions differed significantly with the group A patients (under two and half years of age) doing better.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Double outlet right ventricle associated with persistent common atrioventricular canal and pulmonary stenosis.

A case of a month-old baby admitted for dyspnea and cyanosis, is described. His past history consisted of only mild cyanosis during crying. On physical examination nail beds and lips were cyanotic; on heart auscultation the pulmonary second sound was single. The chest X-ray showed a decreased pulmonary vascularity; the ECG demonstrated extreme right axis deviation (-170 degrees). Capillary gas analysis revealed hypoxic metabolic acidosis. Ten hours after his admission the child suddenly died. The anatomicopathological examination stated the diagnosis of double outlet right ventricle (DORV), infundibular pulmonary stenosis and common atrioventricular canal (transitional type). When clinical findings are compatible with DORV the ECG could be of value in establishing the presence of common atrioventricular canal when extreme right axis deviation is found.

Heart Defects, Congenital↗

Haemangiosarcoma of the pulmonary valve presenting as a pulmonary stenosis. A case report.

Intractable congestive cardiac failure, unexplained arrhythmias, changes in the cardiac silhouette and murmurs which change with position are all features which should arouse suspicion of a cardiac tumour. A case of intracardiac haemangiosarcoma of endothelial origin is discussed. The tumour presented as a pulmonary stenosis and extended into the right ventricular myocardium, causing a right bundle-branch block. At first the tumour seemed to be restricted to the endothelium of the pulmonary vascular bed, but it later infiltrated the interstitium and bronchi, cuasing dyspnoea and haemoptysis. After surgical removal of the tumour and reconstruction of the pulmonary outflow tract, the patient was free of symptoms for 14 months. However, rapid tumour growth in the pulmonary arterial system, lung interstitium and right ventricle subsequently recurred. Deep radiotherapy did not cause the tumour to regress.

Female↗

Successful correction of double-outlet right ventricle with a ventricular D-l-malposition of the great arteries, bilateral conus, pulmonary stenosis and subaortic ventricular septal defect.

The authors present the case of a fifteen-year old girl with double outlet right ventricle with ventricular d-loop and l-malposition of the great arteries, bilateral conus, pulmonary stenosis and subaortic ventricular septal defect, who was operated on successfully. This is the fourth case of double outlet right ventricle with l-position of the aorta that has been surgically corrected. The subaortic position of the interventricular defect favours the creation of the tunnel connecting the left ventricle with the aorta without obstructing the right ventricular outflow tract. The patient was doing well 11 months postoperatively.

Abnormalities, Multiple↗

Transient transcatheter balloon closure of patent foramen ovale following surgical repair of critical pulmonary stenosis.

Severe cyanosis resulting from postoperative atrial right-to-left shunt is a life-threatening complication. We present a technique of transient transcatheter balloon closure of a patent foramen ovale in a newborn operated on for critical pulmonary stenosis, where the foramen ovale had been left open intraoperatively. Cardiac catheterization was performed under echocardiographic control in the intensive care unit and the foramen ovale was occluded with a water-filled balloon-catheter. Significant improvement of arterial oxygen tension allowed delayed definitive surgical closure in a second step. Unfortunately, the child developed right-sided pneumothorax postoperatively and died of cardiopulmonary failure. Nevertheless, this procedure seems a suitable way to relieve atrial right-to-left shunt temporarily until definitive surgical closure can be performed.

Anastomosis, Surgical↗