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CLINICAL RESEARCH: CARDIOMYOPATHY

The prognostic importance of left ventricular outflow obstruction in hypertrophic cardiomyopathy varies in relation to the severity of symptoms

Camillo Autore, MD^_*,_*, Paola Bernabò, MD, Caterina Stella Barillà, MD^_*, Paolo Bruzzi, MD, PhD and Paolo Spirito, MD

^_* Divisione di Cardiologia, Ospedale Sant'Andrea, Università "La Sapienza", Rome
Divisione di Cardiologia, Ente Ospedali Galliera, Genoa
Servizio di Epidemiologia Clinica, Istituto Nazionale Ricerca sul Cancro, Genoa, Italy

Manuscript received May 28, 2004; revised manuscript received November 2, 2004, accepted December 21, 2004.

^_* Reprint requests and correspondence: Dr. Camillo Autore, Università di Roma "La Sapienza", Ospedale Sant'Andrea, Via Grottarossa 1035–1039; 00189 Rome, Italy (Email: camillo.autore{at}uniroma1.it).

OBJECTIVES: The aim of this study was to investigate whether the prognostic importance of left ventricular (LV) outflow obstruction in hypertrophic cardiomyopathy (HCM) is influenced by other predictors of morbidity and mortality.

BACKGROUND: It remains unknown whether the effect of outflow obstruction on clinical outcome in HCM is influenced by other determinants of survival.

METHODS: We assessed the impact of severity of symptoms, LV wall thickness, atrial fibrillation, and age on the prognostic importance of outflow obstruction in a large HCM population.

RESULTS: Of 526 consecutive HCM patients, 141 (27%) had outflow obstruction. During a follow-up of 4.5 ± 4.1 years, 34 patients died of HCM. The incidence of cardiovascular death was significantly higher among patients with LV obstruction than among those without obstruction (relative risk [RR] = 2.14; p = 0.02). The prognostic power of the outflow gradient changed in relation to severity of symptoms (p = 0.024). At initial evaluation, LV obstruction was a significant predictor of cardiovascular mortality only in New York Heart Association (NYHA) functional class I to II patients (hazard ratio [HR] = 2.38; p = 0.025). During follow-up, at time of development of severe symptoms, the outflow gradient lost its prognostic significance (HR = 1.18; p = 0.66), whereas NYHA functional class III to IV was associated with an eight-fold increase in risk of cardiovascular mortality (HR = 7.90; p < 0.001).

CONCLUSIONS: In patients with HCM, the prognostic importance of LV outflow obstruction varies in relation to the severity of symptoms. In patients with mild or no symptoms, obstruction is an important predictor of cardiovascular death. After development of severe symptoms, NYHA functional class becomes the dominant marker of prognosis independently of the presence of an outflow gradient.

Abbreviations and Acronyms   AF = atrial fibrillation   CI = confidence interval   HCM = hypertrophic cardiomyopathy   HR = hazard ratio   LV = left ventricular   NYHA = New York Heart Association

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