Ebstein's anomaly of tricuspid value coexisting with rheumatic mitral stenosis.
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Of 530 patients undergoing tricuspid valve surgery from January 1, 1961 through December 31, 1987, those 362 patients having had preoperative right ventricular angiocardiography were studied. Mean age was 58.5 years. There were 71 males and 291 females. Tricuspid valve replacement (TVR) was performed in 126 (34.8%), and 236 underwent tricuspid valve repair (65.2%). The predominant pathology was combined tricuspid insufficiency and stenosis in 18 patients (4.9%), and isolated tricuspid insufficiency in 344 (95.1%). There were no complications related to the right ventriculogram. Preoperative angiographic severity of tricuspid valve incompetence was grade 1 in 23 patients (6.4%), grade 2 in 65 (17.9%), grade 3 in 109 (30.1%), and grade 4 in 165 (45.6%). Intraoperative assessment of the severity of tricuspid valve incompetence correlated 72% of the time with the preoperative angiographic assessment, in those patients with more severe degrees of incompetence (grade 3+ or 4+), p (X 2) less than 0.001. Increasing severity of preoperative angiographic triscuspid valve incompetence was associated with the requirement for TVR (vs. repair), p (X 2) = 0.0002. In conclusion, preoperative right ventricular angiography is a useful method for assessing tricuspid valve function and may predict the requirement for TVR in patients undergoing tricuspid valve surgery.
A successful palliative arterial switch operation with arch repair in a 30-day-old infant with tricuspid atresia, ventriculoarterial discordance, subaortic stenosis, coarctation of the aorta, and aortic arch hypoplasia is reported. A concomitant Blalock-Taussig shunt was required because of insufficient pulmonary blood flow through the restrictive bulboventricular foramen. Postoperative arterial oxygen saturation was maintained around 90% and the pressure gradient between the left ventricle and the pulmonary artery was 45 mmHg according to a Doppler echocardiogram. The infant becomes free of subaortic stenosis and is awaiting the subsequent Fontan operation. This alternative palliative operation for neonates and early infants with double inlet ventricle, subaortic stenosis, and arch hypoplasia offers some advantages on the Fontan operation for avoiding pulmonary arterial distortion and subaortic stenosis followed by ventricular hypertrophy.
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Most forms of congenital heart disease (CHD) result from aberrations in cardiac morphogenesis including errors in septation, valve formation, and proper patterning of the great vessels. Transcription factors are key proteins that dictate mRNA synthesis rate and subsequent protein production in most eukaryotes. NFATC1 belongs to the Rel family of transcription factors. In mice, it is expressed in the embryonic heart and is restricted to the endocardium where it plays a major role in valve formation. To establish a role for NFATC1 in CHD, we started screening for mutations in the exons encoding the DNA-binding domain of NFATC1 in patients enrolled in our study on CHD in Lebanon. DNA was extracted from patients with pulmonary stenosis (PS), tricuspid atresia (TA) and ventricular septal defect (VSD). PCR amplification and DNA sequencing were done on the patients and their parents and (or) siblings. PCR amplification of the exon 7 region showed that 2 bands are obtained in 57% of patients with CHD (32/56) and in 45% of their healthy parents and (or) siblings. Sequencing of the 2 bands revealed that both are amplicons of the exon 7 region, and that the additional band harbors an additional 44 nucleotides segment in the intronic region. The homozygous form of this allele was only present in patients with VSD (2/21). A screen of a pool of 81 healthy, unrelated individuals showed no presence for the homozygous form of this allele, suggesting that NFATC1 is a potential VSD-susceptibility gene.
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CONTEXT: Cardiac injury has been well recognised as a complication of blunt chest trauma. Its clinical spectrum ranges from blunt cardiac injury (BCI) to complete rupture of cardiac tissues, with cardiac valvular injury often being overlooked. OBJECTIVE: To determine whether hospitalised BCI is associated with increased risk of cardiac valve insufficiency in a large multi-state hospitalised population. METHODS: Cases with BCI and cardiac valve insufficiency were identified based on discharge diagnoses in 1997 statewide hospital discharge data from 19 states. Four valvular outcomes were studied: (1) mitral valve insufficiency, incompetence, regurgitation (MVIIR); (2) aortic valve insufficiency, incompetence, regurgitation, stenosis (AVIIRS); (3) tricuspid valve insufficiency, incompetence, regurgitation, stenosis (TVIIRS); and (4) pulmonary valve insufficiency, incompetence, regurgitation, stenosis (PVIIRS). RESULTS: Among 1,051,081 injury discharges, 2709 (0.26%) people had BCI; 13,087 (1.25%) had MVIIR; 9811 (0.93%) had AVIIRS; 1338 (0.13%) had TVIIRS; 178 (0.02%) had PVIIRS. Independent of potential confounding factors, discharge for BCI was associated with a 12-fold increased risk for TVIIRS and a 3.4-fold increased risk for AVIIRS. CONCLUSION: Cardiac valve insufficiency has been well recognised as an important risk factor for congestive heart failure. With the findings that BCI is associated with an increased risk of specific valvular disorders, it is possible that trauma may play an important and heretofore largely unrecognised role in a portion of the burden of cardiovascular morbidity and mortality.
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