Quadrivalvular rheumatic heart disease

Three cases of chronic rheumatic heart disease with involvement of all four valves are presented. The involvement of tricuspid and pulmonary valves was suspected clinically and was confirmed by two-dimensional echo, Doppler, hemodynamic and angiographic findings. These findings were also verified surgically and histopathologically in 2 cases. One of the cases died after cardiac catheterization; the other 2 cases were treated surgically with success.     

Quadrivalvular heart disease

A case of chronic rheumatic heart disease with quadrivalvular involvement is presented. Pulmonary valve involvement was diagnosed only at surgery. The rarity of this disorder and problems regarding diagnosis and management are highlighted.     

Stent dilatation of pulmonary artery stenosis in the adult patient with congenital heart disease

Stents have been previously used to resolve stenoses of branch pulmonary arteries in children. We report 3 patients, with mean age of 22.7 +/- 4.7 years and pulmonary artery stenosis after palliative surgery in whom we implanted seven stents in four procedures. Six P308 Palmaz, overlapped two by two, were implanted by venous femoral approach in two patients, receiving four in the first case and the other two in the third case. In the second case, a NIR type stent was implanted through femoral artery in the right pulmonary artery. Stenosis diameter enlarged from 5.3 +/- 2.3 to 14.4 +/- 4.2 mm and the pressure gradient through stenosis fell from 40.6 +/- 15.3 to 6. 5 +/- 5 mmHg. All stents are well deployed and there are two patients waiting for total correction (previously not feasible) during a follow-up of 30.6 +/- 6.1 months.     

Ambrisentan for pulmonary arterial hypertension due to congenital heart disease

Eisenmenger syndrome (ES) is a known complication of congenital heart disease associated with unrepaired systemic to pulmonary shunts. Evidence for use of targeted pulmonary arterial hypertension therapy in ES is limited. The early experience using ambrisentan was evaluated in a cohort of consecutive patients with ES who initiated ambrisentan at Columbia University's Pulmonary Hypertension Center from January 1, 2007, to August 1, 2008. Effects of ambrisentan on rest and exercise systemic arterial oxygen saturation (S(a)O(2)), exercise capacity, functional status, hemoglobin levels, and hemodynamics were evaluated and compared using paired Student's t tests. Seventeen patients were evaluated at short-term (mean 163 ± 57 days) and longer term (mean 2.5 ± 0.5 years) follow-up. At short-term follow-up, there was an improvement in exercise capacity (6-minute walking distance 389 ± 74 vs 417 ± 77 m, p=0.03, n=11) and maintenance of rest S(a)O(2) (89 ± 7% vs 89% ± 6%, p=0.75, n=15), exercise S(a)O(2) (75 ± 15% vs 77% ± 15%, p=0.33, n=11), functional class (improvement in 2 patients, no change in 13), and hemoglobin (16.5 ± 2.8 vs 15.8 ± 1.8 g/dl, p=0.11, n=14). At longer term follow-up compared to baseline and short-term follow-up, there was stability of exercise capacity, S(a)O(2), functional class, and hemoglobin. In conclusion, in this single-center cohort of patients with ES, ambrisentan was safe and was associated with increasing exercise capacity at short-term follow-up, with patients maintaining S(a)O(2), functional class, and hemoglobin, and with no significant evidence of clinical deterioration at longer term follow-up. Additional studies are required to further assess the efficacy of ambrisentan in patients with ES.     

Complete heart block after balloon dilatation for congenital pulmonary stenosis

Permanent complete heart block developed in a five year old child during balloon dilatation for pulmonary valve stenosis. Damage to the atrioventricular node by pressure from the inflated balloon may have caused the conduction defect.     

Complete heart block after balloon dilatation for congenital pulmonary stenosis.

Permanent complete heart block developed in a five year old child during balloon dilatation for pulmonary valve stenosis. Damage to the atrioventricular node by pressure from the inflated balloon may have caused the conduction defect.     

Pure congenital pulmonary stenosis: Case report

A case of congenital pulmonary stenosis, with no other associated congenital defects of the heart, is reported. Death was caused by chronic rightsided heart failure at the age of 11 months.     

提高先天性心脏病并发肺动脉高压外科治疗效果的综合方案

目的:探讨先天性心脏病并发肺动脉高压的手术适应证,包括术前综合评价病情、充分降肺动脉压、术中综合处理、术后重症监护,以提高其外科治疗成功率。方法: 对我院2009年1月～2010年12月在全麻低温体外循环下手术治疗室间隔缺损（VSD）并发肺动脉高压（pulmonary hypertension,PH）患者280 例进行回顾性分析。术前综合评价有无手术适应证及充分降肺动脉压准备:所有患者均接受氧疗,经静脉微泵静脉注射扩血管药［硝普纳和（或）前列地尔注射液］,必要时行心导管检查。术中综合处理:术中加强体外循环心肺保护及降肺动脉压的药物应用,精细操作,对部分极重度肺动脉高压的患者留单向活瓣,术中特别注意探查三尖瓣的处理同时矫正其他并发畸形。术后处理:术后呼吸机辅助呼吸时间适当延长,用呼气末正压（PEEP）、过度通气、一氧化氮治疗等。结果: 本组术后死亡5例,病死率1.8%,发生并发症者18 例（发生率9%）。入院后经术前降肺动脉压力及氧疗后,平均肺动脉压力由(71±14) mmHg降至手术开始前的(58±11) mmHg（P<0.05）,手术后肺动脉压力进一步下降至出院前的平均肺动脉压力 (38±5) mmHg（P<0.05）。术后1年复查超声心动图265例,缺损处残余分流2例,三尖瓣中度反流2例、重度返流1例。结论: 我院术前正确评估、积极降压准备、术中综合处理、术后重症监护的综合外科治疗措施,明显提高了先天性心脏病并发肺动脉高压的治疗效果。     

Oral hydralazine in patients with pulmonary vascular disease secondary to congenital heart disease

Two patients with pulmonary vascular obstructive disease secondary to congenital heart disease were evaluated hemodynamically before and during oral hydralazine therapy. Both patients were assessed postoper-atively and had no significant residual shunts. Pulmonary vascular resistance failed to decrease, and an increase in pulmonary arterial pressure occurred because of increased cardiac output secondary to systemic arteriolar dilatation. These responses were sufficiently consistent to warrant a warning against the use of oral hydralazine except under controlled conditions in patients with secondary pulmonary vascular obstructive disease.     

Membranous supravalvular mitral stenosis: a treatable form of congenital heart disease

The clinical data, echocardiographic findings, operative anatomy and postoperative follow-up were assessed in 14 patients who had surgery for membranous supravalvular mitral stenosis between 1978 and 1985. The patients ranged in age from 6 weeks to 13 years at the time of operation, and 8 of the 14 had associated mitral valve abnormalities. Other associated lesions included ventricular septal defect (n = 7), coarctation of the aorta (n = 5), left superior vena cava (n = 6), subaortic stenosis (n = 3) and atrial septal defect (n = 1). Twelve of the 14 patients had successful removal of the supravalvular membrane, which was usually adherent to the valve, and 2 patients with associated mitral valve abnormalities underwent mitral valve replacement. There were no operative deaths. Review of preoperative two-dimensional echocardiograms, which were available in 11 patients, revealed two types of membranous supravalvular mitral stenosis in 10 patients. In four of these patients, the membrane was only evident after repeated stop action viewing from a single subcostal or parasternal location. The membrane was never seen in one patient. Eleven patients had follow-up in excess of 1 year, and there was one late death. Eight of the remaining 10 patients are asymptomatic, and 7 have no clinical evidence of residual mitral obstruction. Failure to recognize membranous supravalvular mitral stenosis can result in undue delay of cardiac surgery with resultant cardiopulmonary deterioration. Patients with evidence of left ventricular inflow obstruction should have extensive echocardiographic evaluation in an effort to detect membranous supravalvular mitral stenosis, which may be amenable to surgical repair.