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1/34. Subaortic obstruction after the use of an intracardiac baffle to tunnel the left ventricle to the aorta.

    Postoperative hemodynamic studies in five patients document subaortic obstruction after surgical repair utilizing an intracardiac baffle to establish continuity between the left ventricle and the aorta. Four of the patients had a Rastelli procedure for D-transposition of the great arteries with a ventricular septal defect and pulmonary stenosis; one patient had repair of double outlet right ventricle with a ventricular septal defect and pulmonary stenosis. The left ventricular outflow was shown to be a long narrow tunnel by angiography in four of five patients and by echocardiography in one patient. Resting aortic peak systolic pressure gradient ranged from 10 to 42 mm Hg (mean 24). The obstruction was localized to the proximal end of the left ventricule to aorta tunnel (i.e., at the site of ventricular septal defect) in five patients. One patient with a gradient of 42 mm Hg has angina and decreased exercise tolerance. Subaortic obstruction is a newly described sequelae after the Rastelli procedure for transposition or repair of double outlet right ventricle. The obstruction may be hemodynamically significant and should be searched for at postoperative cardiac catheterization. ( info)

2/34. 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. ( info)

3/34. Pacing lead adhesions after long-term ventricular pacing via the coronary sinus.

    Left ventricular pacing via the coronary sinus is being increasingly used. There is little data to guide possible lead extractions that might be required in the future. Significant adhesions to the coronary veins were found 12 years after placing a pacing lead in the posterolateral coronary vein in a man with double inlet left ventricle and severe subpulmonary stenosis who had undergone a Fontan operation. The appearances suggest that percutaneous extraction from the proximal coronary sinus may be feasible but that difficulty may be encountered if the lead tip is placed into the distal coronary veins. ( info)

4/34. trisomy 13 with a 13q14q translocation.

    A sporadic case of Patau syndrome with 46,XY,14-,t(13q14q) karyotype is reported in a 2-month-old child. Dermatoglyphic and cytogenetic findings of the propositus and cytogenetic study of his parents are presented. ( info)

5/34. Giant pulmonary artery aneurysm with right ventricular outflow tract obstruction.

    aneurysm of the main pulmonary artery is rare. Its natural history is not well understood and there are no clear guidelines regarding its optimal treatment. We present a case of a huge saccular aneurysm of the main pulmonary artery which was associated with infundibular and valvular pulmonary stenosis. It was repaired using a pericardial patch with concomitant pulmonary valvotomy and infundibular resection. Postoperative recovery was uneventful and the patient is doing well. Follow-up echocardiogram revealed good repair. ( info)

6/34. Surgical correction of unusual double-outlet right ventricle.

    This paper presents the case history of an 8-year-old girl who had total situs inversus and double-outlet right ventricle with pulmonary stenosis and severe tricuspid insufficiency in the presence of dextrocardia with ventricular discordance. A successful repair was performed using the Rastelli technique in conjunction with replacement of the tricuspid valve with a Bjork-Shiley prosthesis. The postoperative course was uneventful, and follow-up catheterization revealed a good operative result. However, the patient died suddenly during an emotionally upsetting period about two months after the operation. Postmortem examination revealed only signs of moderately severe cardiac decompensation. Some anatomical and embryological comments are made. ( info)

7/34. Primitive ventricle with acquired subpulmonary stenosis.

    The course and natural history of two children aged 1 yr and 5 yr with primitive ventricle with outlet chamber and normally related great arteries are described. They initially presented as ventricular septal defect with increased pulmonary blood flow and subsequently developed 'cyanotic attacks', chronic cyanosis and diminished pulmonary flow by acquiring subpulmonary obstruction of the bulboventricular foramen. Both had successful surgical treatment utilizing their own normal pulmonary valves by connecting the right atrium to the right ventricular outflow, with a Dacron conduit in one, and in the other by detaching the pulmonary artery and valve from the rudimentary outflow chamber and anastomosing it directly to the right atrial appendage. Changing morphology and its effect on function are discussed. ( info)

8/34. Subvalvular pulmonary stenosis, demyelination and myelodysplasia with monosomy 7.

    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. ( info)

9/34. A case of congenitally protected d-transposition of the great arteries in a very low-birth-weight infant.

    An arterial switch is the corrective procedure of choice for d-transposition of the great arteries but may be associated with increased morbidity and mortality when performed in low-birth-weight infants. Conversely, delaying surgery often leads to left ventricular "deconditioning" as pulmonary arteriolar resistance decreases. We present an infant with a birth weight of 940 g with d-transposition of the great arteries with an intact ventricular septum whose bilateral pulmonary artery branch stenosis allowed for maintenance of near systemic left ventricular pressure, thereby protecting against deconditioning. This case also represents the smallest reported patient to undergo a successful balloon atrial septostomy. ( info)

10/34. situs inversus totalis associated with subaortic and subpulmonic stenosis.

    The unusual occurrence of total situs inversus and idiopathic hypertrophic subaortic stenosis with the demonstration of right and left heart dynamic obstruction in one patient is presented. The fact that the patient was known to have a rare abnormality (total situs inversus) and the presenting symptom being angina pectoris, may have obscured the diagnosis of IHSS and emphasized the value of comprehensive evaluation of patients. The documentation of right ventricular dynamic obstruction, in addition to obstruction of the left side of the heart, was facilitated by the use of simultaneous pressure recordings in the pulmonary artery and in the body of the right ventricle. The angina-like pain was secondary to left ventricular outflow obstruction, rather than to coronary disease. ( info)
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