Cardiopulmonary malformations in the inv/inv mouse.
(1/14)
The inv/inv mouse carries an insertional mutation in the inversin gene, (inv, for inversion of embryonic turning). Previously it had been reported that almost 100% of the homozygous offspring (inv/inv) were characterized by situs inversus totalis. In this report we identify the spectrum of cardiopulmonary anatomical abnormalities in inv/inv mice surviving to birth to determine whether the abnormalities seen are of the categories classically associated with human situs abnormalities. Stillborn mice, offspring that died unexpectedly (within 48 hr after birth), and neonates with phenotypic characteristics of situs inversus (right-sided stomachs, growth failure or jaundice) were processed for standard histological examination. Of 173 offspring, 34 (20%) neonates (11 stillborn, 9 unexpected deaths, and 14 mice with situs inversus phenotype) were examined, 27 of which were genotyped to be inv/inv. Interestingly, three inv/inv mice (11%) were found to have situs solitus. Twenty-four had situs inversus with normal, mirror-image cardiac anatomy (dextrocardia with atrioventricular concordance, ventriculoarterial concordance and a right aortic arch). The overall incidence of cardiovascular anomalies observed was 10 out of 27 (37%). The most frequent severe malformation, identified in 3 out of 27 animals, was a complex consisting of pulmonary infundibular stenosis/atresia with absence of pulmonary valve tissue and a ventricular septal defect. The pulmonary phenotype in inv/inv mice was situs inversus with occasional minor lobar abnormalities. We conclude that 1) cardiopulmonary malformations in inv/inv mice are not rare (37%), 2) the cardiopulmonary malformations observed in inv/inv specimens are not of the spectrum typically associated with human heterotaxia. In particular, inv/inv mice have a propensity for defects in the development of the right ventricular outflow tract and the interventricular septum, and 3) approximately one out of ten inv/inv mice is born with situs solitus and shows cardiac anomalies that correspond to those observed in inv/inv specimens with situs inversus. Our data therefore suggest that inversin, the product of the inv locus, may have specific roles in cardiac morphogenesis independent of its role in situs determination. (+info)
Dacron conduit with a stented porcine xenograft valve in the anatomic correction of transposition of the great arteries and subpulmonary stenosis.
(2/14)
A stented porcine xenograft valve, sutured into a woven Dacron tubular graft, has been used in the anatomic correction of D-loop, D-transposition of the great arteries, and subpulmonary stenosis. The main advantage of this technique is its simplicity and practicality, as both Dacron tubular grafts and stented porcine xenograft valves are commercially available. (+info)
Characteristics and management of cleft mitral valve.
(3/14)
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. (+info)
Percutaneous transcatheter myectomy of subvalvar pulmonary stenosis in tetralogy of Fallot: a new palliative technique with an atherectomy catheter.
(4/14)
A male infant with tetralogy of Fallot was given a modified left Blalock-Taussig shunt at 11 days of age because of duct dependence. Twenty months later, because of increasing cyanosis and polycythaemia, he was given palliative treatment with balloon dilatation of the right ventricular outflow tract. Seven months later a right ventricular angiogram showed further progression of the infundibular stenosis with poor anterograde opacification of the pulmonary arteries. He underwent percutaneous transcatheter myectomy of the infundibulum with an atherectomy catheter. One month later he was well and less cyanosed. (+info)
Three-vessel coronary artery disease complicated with congestive heart failure in a highly aged patient with tetralogy of Fallot having undergone palliative surgeries.
(5/14)
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. (+info)
Prosthetic aortic valve endocarditis complicated with annular abscess, sub-aortic obstruction and valve dehiscence.
(6/14)
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. (+info)
Imaging congenital heart disease in adults.
(7/14)