The results of operation in patients with hypertrophic cardiomyopathy and pulmonary hypertension.

The clinical course and hemodynamic results in patients undergoing operation for obstructive hypertrophic cardiomyopathy with preoperative pulmonary arterial hypertension were unknown. The hypothesis tested in this retrospective study was that operative relief of left ventricular outflow tract obstruction resulted in a substantial reduction in pulmonary artery pressures and mitral regurgitation without necessitating mitral valve replacement. Patients were included if their preoperative pulmonary systolic pressure was greater than 35 mm Hg and if they were without concomitant cardiac disease, with the exception of mitral regurgitation. Since 1962, 49 patients who fit our criteria underwent left ventricular myotomy and myectomy with 98% follow-up. Mean follow-up was 7.9 +/- 0.7 (mean +/- standard error of the mean) years with a range of 0.8 to 18.4 years. Early hospital mortality rate was 12% (n = 6); two deaths from low cardiac output and four from arrhythmia. There were 43 (88%) hospital survivors and 18 late deaths. Actuarial survival rate after operation was 87% +/- 5% (n = 31) at 5 years and 55% +/- 8% (n = 9) at 10 years. Thirty-nine of 43 survivors (91%) returned 9 +/- 1 months postoperatively for follow-up evaluation including cardiac catheterization. The majority (83%) were in New York Heart Association functional class I or II postoperatively. Cardiac catheterizations indicated a fall in pulmonary arterial systolic pressure from 62 +/- 3 (range = 36 to 105) to 38 +/- 2 (range = 21 to 65) mm Hg (p = 0.0001) with no difference in right atrial pressure or cardiac output. Pulmonary arterial wedge mean pressure decreased from 24 +/- 1 to 16 +/- 5 mm Hg (p = 0.0002) and preoperative mitral regurgitation improved or was abolished in 85% of patients studied (n = 13). Rest and maximal provocable left ventricular outflow tract gradients decreased from 81 +/- 7 and 103 +/- 5 to 14 +/- 3 and 45 +/- 8 mm Hg, respectively (p = 0.0001). Comparison of the above-mentioned patients, operated on since 1982, with a preoperatively matched group who underwent mitral valve replacement in the same interval showed no statistically significant difference in mortality, morbidity, hemodynamic outcome, or functional outcome with a mean follow-up of 2 years. We conclude that a consistent, significant reduction (mean = 40%) in preoperative pulmonary arterial systolic pressure, clinical symptoms, and mitral regurgitation occurs with relief of outflow tract obstruction by left ventricular myotomy and myectomy and that pulmonary hypertension and mitral regurgitation are not indications for mitral valve replacement in these patients.