Novel strategy for treatment of pulmonary arterial hypertension: enhancement of apoptosis.

Advanced pulmonary arterial hypertension is characterized by extensive vascular remodeling that is usually resistant to vasodilator therapy. As the major component of the vascular media, decreased apoptosis of pulmonary arterial smooth muscle cell (PASMC) plays key roles during pulmonary vascular remodeling. Recent studies showed that enhancement of apoptosis of PASMC can reverse pulmonary vascular remodeling and severe pulmonary arterial hypertension. Enhancement of apoptosis of PASMC is becoming a novel strategy to reverse severe pulmonary arterial hypertension. This review analyzes some potential strategies to reverse pulmonary vascular remodeling.

Successful surgical correction of total anomalous systemic venous drainage: a report of 2 cases.

Total anomalous systemic venous drainage is a rare malformation and only limited cases have been reported previously. Here we report two cases of total anomalous systemic venous drainage with successful surgical correction.

Surgical intervention for advanced valvular heart disease in 227 cases.

BACKGROUND: Although the results of surgical treatment in cardiac valve disease continue to improve, the postoperative mortality rate and the rate of complications in patients with advanced valvular heart disease (AVHD) are still very high. We did this retrospective study to summarize the surgical experience of heart valve replacement for patients with AVHD and discuss effective ways to improve the surgical outcome. METHODS: From January 1994 to October 2003, surgical procedures of heart valve replacement were performed on 227 (136 men and 91 women) patients with AVHD in our Department of Cardiothoracic Surgery. The clinical data of all patients were collected and analysed. Patients' age ranged from 10 years to 77 years. In preoperative cardiac function grading, 157 cases were NYHA III and 70 cases NYHA IV. Fifty-one patients had had cardiac operations. The ultrasonic cardiac graphs showed that 145 patients suffered from moderate or severe pulmonary hypertension and 73 had combined giant left ventricle. Mitral valve replacement was performed in 32 cases, aortic valve replacement in 90, tricuspid valve replacement in 1, combined mitral and aortic replacement in 103 and combined mitral and tricuspid replacement in 1. Nineteen patients also received surgical corrections for other minor abnormalities during the operations. A logistic model was established to evaluate the influence of perioperative factors on the mortality rate. RESULTS: The operative mortality rate was 13.2% (30/227). The main causes of death included multiple organ dysfunction syndrome (MODS), low cardiac output syndrome and ventricular fibrillation. From the results of the binary noncounterpart multivariate logistic regression, the following statistically significant factors were found to influence the operative mortality rate: redo operation, age >/= 55 years, preoperative NYHA cardiac function grading, extracorporeal circulation time >/= 120 minutes and postoperative usage of GIK (glucose, insulin and potassium) solution. All factors were risk ones except postoperative application of GIK. The Hosmer-Lemeshow goodness of fit coefficient of this model was 0.976. CONCLUSIONS: The risk factors associated with postoperative mortality rate in the patients with AVHD were redo operation, age >/= 55 years, preoperative NYHA cardiac function grading and extracorporeal circulation time >/= 120 minutes. Postoperative usage of GIK acted as a kind of metabolic therapy and will improve the recovery for patients with AVHD. Active perioperative management and care will play a very important role in reducing the operative risk and improving the short term outcome of surgical treatment for the patients with AVHD.

[Diagnosis and surgical treatment of primary cardiac tumors].

OBJECTIVE: To study the clinical characteristics, diagnosis, and surgical treatment of primary cardiac tumors. METHOD: The clinical features and surgical results in 126 cases (1971-2000) of primary cardiac tumors were analyzed retrospectively. RESULTS: Among the 121 patients with benign tumors were treated surgically, 6 had recurrence of the pathologically confirmed intracardiac myxoma in a period of 1 to 7 years after surgery, and all were cured after reoperation. Follow-up of these patients showed satisfying long-term survival. In the other 5 malignant cases, 1 died during the perioperative period, and the others died of progressive heart failure, cardiac tamponade and cardiac rupture, respectively, within 10 months after the final diagnosis. CONCLUSION: The symptoms of primary cardiac tumor are diverse and complex, and echocardiography, CT, MRI are instrumental for a definite early diagnosis. In case of benign tumors, operation usually produces good results, but for malignant tumors characterized by rapid progression, earlier surgical removal in combination with other therapeutic modalities may improve the prognosis.

Diagnosis and surgical treatment of congenital dextrocardia.

OBJECTIVE: To understand the pathological features of congenital dextrocardia and explore its diagnosis and surgical treatment. METHOD: The clinical data from 32 cases of congenital dextrocardia were collected to understand the major cardiac anomalies and evaluate the diagnostic approaches of this disease. Analysis of the effect of surgical treatment was also conducted in 16 among the 32 cases. RESULTS: Complex anomalies such as transposition of great artery (TGA), single ventricle (SV), common trunk (CMT) formation for the arteries were found in 8 out of 13 mirror-image dextrocardia cases, most of the dextroversion (DV) accompanied by physiological correction of the TGA (C-TGA, 11/17) which often involved ventricle septal defect (VSD) and pulmonary stenosis (PS, 8/11). Another 2 cases diagnosed as isolated dextrocardia (IDC) also suffered TGA combined with complete atrioventricular canal (CAVC) defect. Definite diagnoses of dextrocardia (DC) were derived from X-ray examination, including thoracic-abdominal plain X-ray film, hilar computed tomography, high kV chest plain film or ultrafast CT, echocardiogram and angiography. Fontan operation was performed in 8 cases and anatomical correction in 7 with surgical exploration in 1 case. Thirteen patients survived, among whom 8 had complex cardiac defect and 5 of them received modified Fontan operation or total cavopulmonary connection (TCPC). CONCLUSIONS: Complex cardiac anomalies are not rare in mirror-image dextrocardia, and dextroversion and isolated dextrocardia were usually related to C-TGA+VSD+PS and TGA+CAVC respectively. X-ray examinations are of great significance in the diagnoses and classification of dextrocardia, and echocardiogram and ultrafast CT constitute the major modalities for clarifying intracardial defect and surgical indications. Fontan operations, including modified Fontan and total cavopulmonary connection, might be hopeful surgical treatment for complex congenital heart disease when indications are carefully evaluated.

Molecular mechanisms of congenital heart disease.

BACKGROUND: Congenital heart disease (CHD) is the most common type of birth defect. Despite the many advances in our understanding of cardiac development and many genes related to cardiac development identified, the fundamental etiology for the majority of cases of congenital heart disease remains unknown. METHODS: This review summarizes normal cardiac development, outlines the recent discoveries of the genetic causes of CHD, and provides possible strategies for exploring them. RESULTS: CHD is a multifactorial complex disease, with environmental and genetic factors playing important roles. A number of causative genes of selected congenital heart defects and genetic syndromes have been found. The molecular mechanisms of CHD may include mutations in components of the cardiac gene network, altered haemodynamics, regulatory pathway of cardiac genes, micro-RNA dysfunction, epigenetics, adult congenital heart diseases, and so on. CONCLUSIONS: The molecular basis of CHD is an exciting and rapidly evolving field. The continuing advances in the understanding of the molecular mechanisms of CHD will hopefully result in improved genetic counseling and care of affected individuals and their families.