Infundibular pulmonary stenosis after radiotherapy for breast cancer.

Postoperative radiotherapy for breast cancer may be associated with cardiotoxicity. We present a case of acquired infundibular pulmonary stenosis that developed 43 years after radiotherapy for left breast cancer, which necessitated a right ventricular outflow tract myectomy.

A classical case of the Gasul phenomenon.

This case demonstrates the development of secondary infundibular stenosis in a 10-year-old male child with documented large non-restrictive perimembranous ventricular septal defect in infancy - the classical Gasul phenomenon.

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.

Prosthetic aortic valve endocarditis complicated with annular abscess, sub-aortic obstruction and valve dehiscence.

We present a 76-year-old woman with infective endocarditis of a prosthetic aortic valve. The course of her illness started with an ischaemic stroke and she was admitted with prolonged fever and an episode of loss of consciousness. Echocardiography revealed acute aortic regurgitation and dehiscence of the prosthetic valve with excessive "rocking motion", aortic abscesses and left ventricular outflow obstruction caused by a semilunar shelf of tissue probably due to endocarditis vegetations. She underwent an urgent surgical procedure that confirmed the echocardiographic findings. Our case report reinforces the value of early diagnosis in the presence of a high clinical suspicion of prosthetic valve endocarditis. An extended workup, including transoesophageal echocardiography, in such a patient with a mechanical valve is mandatory.

Autologous pericardium patch aneurysm after ventricular septal defect closure and arterial switch operation.

A neonate with L-transposition of the great arteries with ventricular septal defect underwent complete repair using fresh autologous pericardium to close the ventricular septal defect as well as to reconstruct the neo pulmonary artery sinuses. Four months later, the child came back with right ventricular inflow obstruction related to aneurysmal pericardial patch, severe tricuspid regurgitation, and severe supra-valvular pulmonic stenosis. At reoperation, there was a redundant, aneurysmal pericardial patch densely adherent to the septal and posterior leaflets of the tricuspid valve, which was damaged. The pericardial patch was replaced, the pulmonary artery enlarged, and tricuspid valve repaired. Postoperative course was uneventful, but residual moderate tricuspid regurgitation required intensive medical treatment.

Three-vessel coronary artery disease complicated with congestive heart failure in a highly aged patient with tetralogy of Fallot having undergone palliative surgeries.

An increasing number of patients with tetralogy of Fallot (TOF) are reaching older age. We encountered a 75-year-old woman with uncorrected TOF and concomitant severe coronary artery disease (CAD) with congestive heart failure. Her CAD risk factor was hyperlipidemia, which had been untreated. Successful percutaneous coronary interventions have improved her clinical condition and provided long-term survival. Although CAD is considered to be a rare complication in adults with TOF, both strict modification of CAD risk factors and early detection of CAD would be also required in this population, given the residual TOF lesions relating to acute exacerbation of clinical presentation.

Late distortion of the original Palmaz stent implanted in postoperative lesions associated with congenital heart disease.

The objective of this study was to report late distortion of a Palmaz stent. Late distortion of an original Palmaz stent, implanted in an extracardiac lesion, is rare. We completed a 1-year follow-up of 54 patients who had been implanted with 80 Palmaz stents in extracardiac lesions. Distortion of two stents was detected in two patients. For case 1, we implanted a P188 stent for supravalvar pulmonary stenosis complicating an arterial switch operation in a 14-year-old girl. Seven months later, we found compression of the stent. Although we implanted two P308 stents anterior to the distorted stent, distortion of both stents developed after 1 month. Two more P308 stents placed inside each stent were gradually recompressed. A CAT scan showed compression of the stent by a dilated sinus of valsalva. For case 2, we implanted a P308 stent for stenosis of the superior vena cava after Williams operation in an 11-year-old boy. A chest X-ray documented longitudinal compression of the stent 27 months after implantation and a CAT scan showed the ascending aorta was in contact with the stent. A Palmaz stent may be distorted when implanted in a lesion adjacent to a pulsating aorta.

Characteristics and management of cleft mitral valve.

OBJECTIVES: We sought to highlight the clinical, morphologic, and pathogenetic features in patients with a cleft mitral valve (MV). BACKGROUND: Few studies have addressed the morphologic features of cleft MV and the outcome of these patients. The pathogenetic features, including the developmental relation to an atrioventricular (AV) septal defect, remain unclear. METHODS: We reviewed the patients with cleft MV that were diagnosed by echocardiography since 1980. Patients with an AV canal, ventriculo-arterial discordance, and hypoplastic ventricles were excluded. RESULTS: Twenty-two patients were identified at a median age of 0.5 years (range 0 to 10.6). In three patients, no chordal attachments of the cleft to the ventricular septum were seen. Ten patients had significant mitral regurgitation (MR), and three had subaortic obstruction by the cleft. Associated cardiac lesions and extracardiac features were present in 13 and 10 patients, respectively. During the median follow-up period of 1.5 years (range 0 to 11.8), two patients died of extracardiac causes, and one neonate died of severe subaortic obstruction. Surgical repair was performed in 10 patients at a median age of 5.2 years (range 1.3 to 10.6). Multivariate analysis showed no predictors for MV surgery. One patient was re-operated for mitral stenosis associated with aortic valve stenosis. Follow-up echocardiography demonstrated moderate MR in two unoperated patients and moderate MV stenosis in two operated patients. CONCLUSIONS: A cleft of the MV comprises a wide spectrum. Important morphologic differences exist with an AV septal defect, although the two lesions may be pathogenetically related. Surgical repair always seems possible. Long-term echocardiographic follow-up is warranted.

[Incidence and treatment of reactive infundibular obstruction after balloon dilatation of critical pulmonary valve stenoses]. / Inzidenz und Behandlung der reaktiven infundibulären Obstruktion nach Ballondilatation von kritischen Pulmonalstenosen.

Seventeen consecutive newborn and premature babies with critical pulmonary stenosis underwent a technically successful balloon valvuloplasty at our institution from March 1991 to February 1998. The only major complication was a thrombosis of the femoral vein in one patient, causing no clinical problems. Four patients (24%) showed a reactive infundibular obstruction after balloon valvuloplasty. The outflow tract obstruction became evident immediately after successful dilatation of the pulmonary valve with persistently high pressures in the right ventricle. Pathognomonic was a typical notch in the ascending part of the right ventricular pressure curve. We were unable to predict this reaction based on echocardiography or angiography. To relieve the muscular subvalvar obstruction, we treated the first two patients with i.v. Propranolol (0.05 mg/kg over 2 min). The last 2 patients received Esmolol (0.5 mg/kg over 2 min followed by a continuous infusion with 100 micrograms/kg/min), a very short acting beta-blocker. In the medium-term follow-up, all 17 patients had a very good result with only mild pulmonary valve regurgitation. All 4 patients with a reactive infundibular obstruction required no repeat intervention. In the medium-term follow-up there were no differences between these 4 patients and the whole group.

Growth of the neopulmonary valve annulus after arterial switch operation in transposition of the great arteries.

BACKGROUND: It is known that supravalvular pulmonary artery stenosis can occur in patients with d-transposition of the great arteries (TGA) after arterial switch operation (ASO). However, little is known about the growth of the neopulmonary valve annulus after the ASO. This study investigated the growth potential of the neopulmonary (old aortic) valve annulus. METHODS AND RESULTS: Annular diameters of the old aortic and neopulmonary valve were measured from cineangiograms in patients who underwent cardiac catheterizations both before and > 1 year after the ASO. Of 71 patients, 13 (18%) had either a small annulus (< 70% of the expected normal value) or no significant growth of the neopulmonary annulus after the ASO, and 4 (6%) had a pressure gradient of > 30 mm Hg across the valve. The small annulus or no growth of the neopulmonary valve was more frequent in patients with a history of pulmonary artery banding. After the ASO, the valve diameter in patients with a ventricular septal defect was 80 +/- 15% of normal (n = 24), and the value was significantly less than in patients with an intact ventricular septum (91 +/- 11%, n = 47). In all patients with an intact ventricular septum who underwent the one-stage ASO, the valve diameters before and after the ASO were within normal limits, and a significant increase in the pulmonary valve annulus was observed. CONCLUSIONS: These data indicate that not only supravalvular pulmonary stenosis but also pulmonary valvular stenosis due to a small annulus can occur in TGA, especially in patients with a history of pulmonary artery banding and in patients with ventricular septal defect.

Accurate diagnosis of metastatic cardiac leiomyosarcoma with infundibular stenosis and cardiac tamponade by transesophageal echocardiography and Gd-DTPA magnetic resonance imaging--report of a case.

We present a patient with metastatic cardiac leiomyosarcoma that was diagnosed antemortem by transesophageal echocardiography (TEE) and ECG-gated magnetic resonance imaging with gadolinium diethylene triaminepentaacetic acid (Gd-DTPA MRI). TEE and Gd-DTPA MRI clearly revealed the morphological characteristics of infundibular stenosis and cardiac tamponade. Our patient has survived about 30 months after cardiac surgery. Since sarcoma is usually associated with a very poor prognosis, surgical treatment should be performed as soon as possible. TEE with color Doppler imaging and Gd-DTPA MRI are very useful diagnostic methods for determining the precise anatomical characterization of cardiac tumor, and these procedures are prerequisite to precise therapy.