Background Microvascular dysfunction, reflected by an inadequateincrease in myocardial blood flow in response to dipyridamoleinfusion, is a recognized feature of hypertrophic cardiomyopathy.Its long-term effect on the prognosis is unknown. We prospectivelyevaluated a cohort of patients with hypertrophic cardiomyopathyafter they had undergone quantitative assessment of myocardialblood flow by positron-emission tomography (PET).
Methods Fifty-one patients (New York Heart Association classI or II) were followed for a mean (±SD) of 8.1±2.1years after PET. Twelve subjects with atypical chest pain servedas controls. Measurement of flow was performed at base lineand after the infusion of the coronary vasodilator dipyridamole,with the use of nitrogen-13labeled ammonia. Patientswere then divided into three equal groups with increasing valuesof myocardial blood flow.
Results The response of myocardial blood flow to dipyridamolewas severely blunted in the patients, as compared with the controls(1.50±0.69 vs. 2.71±0.94 ml per minute per gramof tissue, P<0.001). Sixteen patients (31 percent) had anunfavorable outcome (death from cardiovascular causes, progressionto New York Heart Association class III or IV, or sustainedventricular arrhythmias requiring the implantation of a cardioverterdefibrillator)2.2 to 9.1 years after PET. Reduced blood flow in response todipyridamole was strongly associated with an unfavorable outcome.Multivariate analysis showed that among patients in the lowestof the three flow groups the age-adjusted relative hazard ofdeath from cardiovascular causes was 9.6 (P=0.02) and the relativehazard of an unfavorable outcome (a combined end point) was20.1 (P=0.003), as compared with patients in the two other flowgroups. Specifically, all four patients who died from heartfailure and three of five who died suddenly were in this subgroup.
Conclusions In patients with hypertrophic cardiomyopathy, thedegree of microvascular dysfunction is a strong, independentpredictor of clinical deterioration and death. Severe microvasculardysfunction is often present in patients with mild or no symptomsand may precede clinical deterioration by years.
Source Information
From the Regional Referral Center for Myocardial Diseases, Azienda Ospedaliera Careggi, Florence (F.C., I.O., R.G.); the Cardiology Unit, Ospedale di Lucca, Lucca (R.L.); the Cardiology Unit, Ospedale di Pescia, Pescia (G.C.); the Consiglio Nazionale delle Ricerche Institute of Clinical Physiology, Pisa (P.G.C.) all in Italy; and the Medical Research Centre, Hammersmith Hospital, Imperial College, London (P.G.C.).
Address reprint requests to Dr. Cecchi at Via Jacopo Nardi 30, 50132 Florence, Italy, or at franco.cecchi{at}asf.toscana.it.
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