PURPOSE: Eisenmenger’s syndrome (ES) refers to the most advanced form of pulmonary arterial hypertension(PAH) associated with congenital unrepaired systemic-to-pulmonary cardiac shunts(CHD). In contrast to other subgroups of PAH, patients with ES are known to exhibit a far better survival. However, we believe this changes significantly once clinical signs of right ventricular failure ensue. Therefore we sought to evaluate the overall survival compared to that of patients with Idiopathic PAH(IPAH).
METHODS: We prospectively followed 33 patients (mean age 12± 5years) diagnosed with CHD or IPAH, from 2000 till February 2010. Cox-proportional hazards regression was used to address association with mortality. Survival curves were derived using the Kaplan-Meier method, and compared with Log-rank or Tarone-Ware accordingly. Numerical values are expressed as mean± SD. Results are expressed as CHD VS IPAH. Statistical significance was accepted as p≤ 0.05.
RESULTS: Hemodynamic profiles were similar for both groups. Right atrial pressure 7± 4.22mmHg VS 10± 5.7(p=0.097), mean pulmonary pressure 73± 17mmHg VS 62± 28(p=0.148), cardiac index 3.37± 1.33 L/min/m² VS 3.27± 1.26(p=.802). Mean survival was 53 mo(CI.95% 40-66) VS 50 mo(37-63).No significant difference was found between groups.For the IPAH group right-sided S3 (HR=5.44),lower extremities edema(HR=5.43), WHO-class(HR=3.511), serum creatinine(HR=4.524), uric acid (HR=4.03) and cardiac index(HR=0.186) were significantly related to mortality. Nevertheless, none of them were independent predictors. Thirty-three percent of all patients died. Most frequent cause of death was PAH progression, although a very high number died of massive hemorrhage.
CONCLUSION: We conclude that once right ventricular failure becomes clinically apparent, and symptoms are unbearable enough to force the patient seek for medical attention, the prognosis is poor. Even for the formerly adapted hearts of ES patients.
CLINICAL IMPLICATIONS: It is true that PAH-therapies directed toward vasoconstriction and vascular remodeling have improved the quality of life of many patients with severe PAH, however, afterload reduction cannot be achieved in many cases. Therefore, the transitory adaptation to pressure overload eventually crosses the diffuse juncture between compensatory remodeling and heart failure.
DISCLOSURE: Jose Gomez-Arroyo, No Financial Disclosure Information; No Product/Research Disclosure Information