Impact of sildenafil therapy on pulmonary arterial hypertension in adults with congenital heart disease.
ABSTRACT It has been demonstrated that sildenafil is effective in patients with pulmonary arterial hypertension (PAH). However, the impact of sildenafil on PAH in adults with congenital heart disease (CHD) has been less investigated.
In this prospective, open-label, uncontrolled and multicenter study, 60 patients with PAH related to CHD received oral sildenafil (75 mg/day) for 12 weeks. The enrolled patients underwent six-minute walk test (SMWT) and cardiac catheterization at the beginning and the end of the 12 weeks. The primary end point was the changes in exercise capacity assessed by SMWT; the secondary end point included assessment of functional class, evaluation of cardiopulmonary hemodynamics, and clinical worsening (defined as death, transplantation, and rehospitalization for PAH). Drug safety and tolerability were also examined.
Oral sidenafil significantly increased SMWT distances (422.94 ± 76.95 m vs. 371.99 ± 78.73 m, P < 0.0001). There was also remarkable improvement in Borg dyspnea score (2.1 ± 1.32 vs. 2.57 ± 1.42, P = 0.0307). Moreover, significant improvements in World Healthy Organization (WHO) functional class and cardiopulmonary hemodynamics were also discovered (mean pulmonary artery pressure, P = 0.0002; cardiac index, P < 0.0001; pulmonary vascular resistance, P < 0.0001). Side effects in this study were mild and consistent with reported studies. None of the enrolled patients experienced significant clinical worsening.
This study confirmed and extended previous studies. It suggested that oral sildenafil was safe and effective for the treatment of adult patients with CHD-related PAH.
- Pediatric Research 10/1986; 20(9):811-24. · 2.67 Impact Factor
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ABSTRACT: We examined the cases of 702 patients found to have isolated atrial septal defect of the secundum or sinus venosus type at catheterization from 1953 to 1978. Forty patients (6%), 34 women and six men, had pulmonary vascular obstructive disease, with a total pulmonary resistance greater than 7 U/m2; of these patients 26 (mean age 47 years) underwent surgical closure and 14 (mean age 44 years) received medical treatment. All patients were followed for at least 4 years, with a median follow-up of 12 years. At the most recent follow-up, 17 of the 40 patients were dead. Of the 22 surgically treated patients with total pulmonary resistance less than 15 U/m2, 19 were alive with significant regression of symptoms. All four surgically treated patients with total pulmonary resistance greater than or equal to 15 U/m2 were dead. Of the five medically treated patients with total pulmonary resistance less than 15 U/m2, four had died, and one was alive with significant progression of symptoms. Of the nine medically treated patients with total pulmonary resistance greater than or equal to 15 U/m2, six had died and the three survivors had progression of symptoms. In the surgically treated group, the following variables correlated with survival: total pulmonary resistance (p less than .00001), pulmonary arteriolar resistance (p less than .00001), pulmonary-to-systemic resistance ratio (p = .004), systemic arterial oxygen saturation (p = .005), and pulmonary arterial oxygen saturation (p = .007). In conclusion: (1) Atrial septal defect with high total pulmonary resistance is uncommon and predominates in adult female patients.(ABSTRACT TRUNCATED AT 250 WORDS)Circulation 12/1987; 76(5):1037-42. · 15.20 Impact Factor
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ABSTRACT: A retrospective study of adults with Eisenmenger syndrome assessed at a tertiary referral center was performed to identify clinical characteristics and establish prognostic determinants. Mortality and cause of death are reported with baseline clinical data correlated with mortality to identify predictors of death. Clinical events during follow-up, including heart failure, atrial arrhythmia, hemoptysis, and syncope were also reviewed. A total of 109 adults with Eisenmenger syndrome (mean +/- SD age 29 +/- 11 years, 43% men) were followed for a median of 6.3 years. Sixty-six patients (61%) had simple cardiac anatomy (13 atrial septal defect, 43 ventricular septal defect, 10 patent ductus arteriosus). The remainder (43 patients) had complex cardiac anatomy (including atrioventricular septal defect, truncus arteriosus, univentricular heart, and transposition of the great arteries). There were 33 deaths and 9 transplantations during follow-up. Median survival was 53 years. Multivariate Cox regression analysis identified age at presentation (hazard ratio [HR] 0.90), supraventricular arrhythmia (HR 3.44), precordial electrocardiogram voltage (HR 1.61/mV increase), and poor New York Heart Association functional class (HR 2.60) as independent predictors of mortality. There is a large variation in the life expectancy for adults with Eisenmenger syndrome. Baseline characteristics associated with increased mortality include younger age at presentation (associated with complex anatomy), functional class, supraventricular arrhythmia, and an electrocardiogram index for right ventricular hypertrophy.The American Journal of Cardiology 10/1999; 84(6):677-81. · 3.21 Impact Factor