Background Sudden death is a possible consequence of hypertrophiccardiomyopathy. Quantification of the risk of sudden death,however, remains imprecise for most patients with this disease.
Methods We assessed the relation between the magnitude of leftventricular hypertrophy and mortality in 480 consecutive patientswith hypertrophic cardiomyopathy. The patients were categorizedinto five subgroups according to maximal wall thickness: 15mm or less, 16 to 19 mm, 20 to 24 mm, 25 to 29 mm, and 30 mmor more. Their ages ranged from 1 to 89 years (median, 47).
Results Over a mean follow-up period of 6.5 years, 65 of the480 patients (14 percent) died: 23 suddenly, 15 of heart failure,and 27 of noncardiac causes or stroke. The risk of sudden deathincreased progressively and in direct relation to wall thickness(P=0.001), ranging from 0 per 1000 person-years (95 percentconfidence interval, 0 to 14.4) for a wall thickness of 15 mmor less to 18.2 per 1000 person-years (95 percent confidenceinterval, 7.3 to 37.6) for a wall thickness of 30 mm or moreand almost doubling from each wall-thickness subgroup to thenext. The cumulative risk 20 years after the initial evaluationwas close to zero for patients with a wall thickness of 19 mmor less but almost 40 percent for wall thicknesses of 30 mmor more. As compared with the other subgroups, patients withextreme hypertrophy were the youngest (mean age, 31 years),and most (41 of 43) had mild symptoms or no symptoms; of the12 patients who were less than 18 years old at the initial evaluation,5 died suddenly.
Conclusions In hypertrophic cardiomyopathy, the magnitude ofhypertrophy is directly related to the risk of sudden deathand is a strong and independent predictor of prognosis. Youngpatients with extreme hypertrophy, even those with few or nosymptoms, appear to be at substantial long-term risk and deserveconsideration for interventions to prevent sudden death. Themajority of patients with mild hypertrophy are at low risk andcan be reassured regarding their prognosis.
Source Information
From the Divisione di Cardiologia, Ente Ospedaliero Ospedali Galliera, Genoa (P.S.); the Divisione di Cardiologia, Ospedale Santa Corona, Pietra Ligure, Savona (P. Bellone); the Cattedra di Cardiologia, Università degli Studi, Genoa (P. Bernabò); the Servizio di Epidemiologia Clinica, Istituto Nazionale per la Ricerca sul Cancro, Genoa (P. Bruzzi) — all in Italy; and the Minneapolis Heart Institute Foundation, Minneapolis (K.M.H., B.J.M.).
Address reprint requests to Dr. Spirito at the Divisione di Cardiologia, Ente Ospedaliero Ospedali Galliera, Via Volta 8, 16128 Genoa, Italy, or at p.spirito{at}galliera.it.
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