Efficacy of sildenafil as a rescue therapy for patients with severe pulmonary arterial hypertension and given long-term treatment with prostanoids: 2-year experience.

BACKGROUND: Both prostanoids and sildenafil are effective treatments for severe pulmonary arterial hypertension (PAH). The combined use of these drugs to maximize the clinical benefit is an emerging treatment option. This study describes a 2-year experience with adjunct sildenafil as a rescue therapy for patients with severe PAH treated long term with prostanoids and who showed clinical deterioration or onset of heart failure. METHODS: Twenty patients (11 men, 9 women; mean age 42 +/- 11 years) with severe PAH, who showed clinical or functional worsening despite ongoing treatment with prostanoids (8 subcutaneous, 7 intravenous, 5 inhaled), were started on adjunct oral sildenafil. New York Heart Association (NYHA) functional class, 6-minute walking test, signs of right ventricular failure and echocardiography were assessed before and after 1 and 2 years of combined therapy. RESULTS: There was a significant improvement of NYHA functional class and signs of right heart failure after 1- and 2-year follow-up. Patients showed a mean increase in 6-minute walking distance of 79 m and 105 m after 1 and 2 years of adjunct sildenafil, respectively. Two patients died during follow-up. The echocardiographic parameters showed a significant reduction of right ventricular end-diastolic diameter and left ventricular diastolic eccentricity index. No serious side effects related to sildenafil were observed. CONCLUSIONS: Adjunct sildenafil to long-term prostacyclin therapy in patients with severe PAH provided sustained clinical stabilization and an improved clinical situation, exercise capacity and echocardiographic parameters of right ventricular function. The beneficial effects were strong and lasted >24 months.

Systolic left atrial failure in elderly women with severe aortic stenosis: mitral and pulmonary vein Doppler analysis by transesophageal echocardiography.

We studied prospectively 35 elder women aged 65-82 years, with isolated severe symptomatic aortic stenosis, referred for aortic valve surgery. We assessed diastolic function by TEE before and after cardiac surgery, although follow-up data were collected in 26 patients. The examination was performed prior to surgery and 6 months after. The control group consisted of 32 patients referred for TEE. In the preoperative study, the velocities and integrals of the waves in the pulmonary vein flow were similar to the people of their same age, except the A-wave of atrial contraction and the integral of the systolic wave, which were significantly smaller (Control A-wave 26.1 +/- 5.1 vs preoperative A-wave 22.6 +/- 5.6, P = 0.009 and control double product A vel xA dur 2,748 +/- 835 vs preoperative 2,273 +/- 968, P = 0.03; systolic integral 14.6 +/- 3.8 vs 11.3 +/- 4, P = 0.0009). Six months after surgery, the PV flow was similar to the control group except for the wave of atrial contraction, which was significantly smaller but tended to normalization (postoperative A-wave 23.3 +/- 5, P = 0.04 vs control, and postoperative double product A vel x A dur 2460 +/- 893, P = 0.21 vs control). Mitral flow parameters did not change in the preoperative and postoperative period. Left ventricular mass index changed from 166 +/- 54 g/m(2) to 105 +/- 39 g/m(2) (P< 0.0001). The results of this study show that in elderly women with symptomatic severe AS, diastolic function does not change, left ventricular mass reduces, with improvement in symptoms, and the left atrium function, considered by pulmonary vein flow, is preoperative depressed and tends to mild recovery in the postoperative period, suggesting systolic LA failure.

Hypertrophic cardiomyopathy associated with left ventricular aneurysm and normal coronary arteries: Case study indicating genetic tendencies of cardiomyopathy.

A 50-year-old man presented with hypertrophic obstructive cardiomyopathy (HOC) associated with a left ventricular aneurysm and normal coronary arteries. His history revealed no evidence of myocardial infarction or atypical angina. Physical examination disclosed HOC but did not suggest the presence of an aneurysm. Although the patient was treated medically, heart failure ensued, and he died suddenly while working his farm. Subsequent investigation of the patient's family revealed that three of his five children were also affected by cardiomyopathy, which was especially pronounced in the eldest, a 22-year-old man. The possible hemodynamic relationship between HOC and left ventricular aneurysm is discussed, along with probable indications. The role of left ventricular aneurysm is also presented in relation to the natural history of the disease.