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Pulmonary valve stenosis is the most frequent congenital heart disease associated with Leopard syndrome, an inheritable condition with a low incidence that we should be aware of and diagnose early. We describe the evolution of a patient with Leopard Syndrome and pulmonary subvalvular stenosis, from birth to the surgical treatment.
Isolated subvalvular pulmonary stenosis is a rare condition and its morphological evaluation is obscure. Whole heart magnetic resonance imaging (MRI) is a new, totally non-invasive technique which allows three-dimensional comprehension of the cardiac structure. We describe a patient with isolated subvalvular pulmonary stenosis, in whom whole heart MRI was useful to detect and evaluate the right ventricular outflow obstruction.
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We report a 23-year-old male with subvalvular pulmonary stenosis (PS) presenting with concurrent acute demyelinating neuropathy and myelodysplasia with monosomy 7. There was no stigmata of Noonan syndrome or neurofibromatosis, nor any family history of cardiac or hematologic disorders. There are 40 reported pediatric cases of Noonan syndrome with subvalvular PS that developed leukemia, over two-thirds with monosomy 7. There is also a genetic overlap between Noonan syndrome and neurofibromatosis, which is also closely associated with demyelination and leukemia. The importance of recognition of rare clinical syndromes that may predispose to leukemia is discussed.
A 22-year-old male who had Mustard repair for complete transposition presented with progressive exercise intolerance, documented by a gradual decrease in exercise capacity on cycle ergometer. Cardiac catheterization and angiography demonstrated subvalvular pulmonary stenosis. After surgical relief of the obstruction, exercise capacity returned to previous level.
A case is reported of right ventricular outflow obstruction caused by hypertrophic cardiomyopathy. The pressure gradient between the inflow tract and the outflow tract of the right ventricle was 84 mm Hg. Resection of the hypertrophic muscle bundles practically eliminated the obstruction leaving only an insignificant subvalvular gradient of 2 mm Hg. Histologic examination revealed cardiac muscle cell hypertrophy and disorganization.
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Congenital corrected transposition of the great arteries in which there is both an atrio-ventricular and a ventriculo-arterial discordance has surgical significance only in consequence of the associated cardiac anomalies. Their surgical correction is subject to multifarious problems. An exceedingly rare cause of a left-ventricular outflow tract obstruction erroneously interpreted preoperatively in an eight-year-old boy is reported. In an l-transposition with inversion of both ventricles, the diagnosis "valvular pulmonary stenosis" was made preoperatively. However, a normally arranged tricuspid pulmonary valve without stenosis was revealed intraoperatively. On the other hand, dystopic valvular tissue in the form of a monocuspid atrioventricular valve which was attached to an accessory papillary muscle with several tiny tendons was found below the pulmonary valve in the morphological left ventricle. The accessory valve cusp, which was the cause of the hemodynamically relevant subvalvular pulmonary stenosis, had no connection with the mitral valve. The abnormal valve cusp was resected without injuring the normal structures. The angiographic follow-up after one year no longer revealed any pressure gradient. This case report points out the possibility of an outflow tract obstruction caused by a complete accesory valve cusp.
Twenty-eight patients younger than age 40 years, treated for Hodgkin's disease with mediastinal irradiation, were examined no less than 5 years after the irradiation in order to evaluate the frequency of cardiac abnormalities. Twelve patients (43%) had had some pericardial event after radiation: a diagnosed pericarditis, remarkably increased heart volume, or a conspicuous change of cardiac silhouette, suggesting pericardial fluid. On evaluation, 50% of the patients complained of symptoms, and 13 patients had to stop the exercise test on a low level because of chest pain, dyspnea, or general fatigue. In 13 patients some of the following abnormalities in the electrocardiogram (ECG) was found: right bundle branch block (four), first-degree atrioventricular block (four), abnormal P terminal force (five), or a low voltage (two). In ten patients (38%) an increase of the pericardial fluid was seen in the echocardiogram, and in nine patients the right ventricle wall thickness had increased. In two patients a severe coronary artery disease was found. One died suddenly after an acute myocardial infarction (AMI), and the other had a large anterior AMI. Two patients with chronic pericardial fluid underwent partial pericardectomy. Two cases of mild pulmonary valve stenosis, one pulmonary subvalvular stenosis and two aortic valve deformities were discovered. Eight patients went through cardiac catheterization, and in all but one case the pressures were slightly elevated suggesting diminished diastolic compliance. In summary, 19 of 28 patients had some abnormal cardiac findings, but only three of them were serious ones.
The pulmonary valve and left ventricular outflow tract of 214 hearts with d-transposition of the great arteries (d-TGA) were visually inspected (126 of 214 with intact ventricular septum and 88 of 214 with ventricular septal defect [VSD]). Pulmonary stenosis (PS), either valvular, subvalvular, or in combination, was present in 26 cases and was found to be more common in the presence of a VSD (20.5%) than intact ventricular septum (6.3%). PS occurred more commonly in the presence of a supracristal VSD than an infracristal VSD (70%, 7 of 10 vs 15%, 10 of 66). Further, infracristal or supracristal VSDs were associated with different types of pulmonary obstruction. In seven of ten cases with infracristal VSD and PS, the stenosis was caused by an anomaly of an atrioventricular valve. In six of seven cases with supracristal VSD and PS, the latter was caused by membranous encirclement of the left ventricular outflow tract.
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