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CLINICAL STUDY: CARDIOMYOPATHY AND PREGNANCY

Risk associated with pregnancy in hypertrophic cardiomyopathy

Camillo Autore, MD^*,*, Maria Rosa Conte, MD, Marco Piccininno, MD, Paola Bernabò, MD, Giovanna Bonfiglio, MD, Paolo Bruzzi, MD, PhD and Paolo Spirito, MD, FACC

^* Dipartimento di Scienze Cardiovascolari e Respiratorie, Università La Sapienza, Rome, Italy
Divisione di Cardiologia, Ospedale degli Infermi, Rivoli, Torino, Italy
Servizio di Epidemiologia Clinica, Istituto Nazionale per la Ricerca sul Cancro, Genoa, Italy
Divisione di Cardiologia, Ente Ospedaliero Ospedali Galliera, Genoa, Italy

Manuscript received April 16, 2002; revised manuscript received June 20, 2002, accepted July 24, 2002.

^* Reprint requests and correspondence: Dr. Camillo Autore, Dipartimento di Scienze Cardiovascolari e Respiratorie, Università di Roma "La Sapienza," Viale del Policlinico 155, 00161, Rome, Italy.
camillo.autore{at}uniroma1.it

OBJECTIVES: We sought to assess mortality and morbidity in pregnant women with hypertrophic cardiomyopathy (HCM).

BACKGROUND: The risk associated with pregnancy in women with HCM is an important and increasingly frequent clinical issue for which systematic data are not available and a large measure of uncertainty persists.

METHODS: Maternal mortality in 91 consecutively evaluated families with HCM was compared with that reported in the general population. The study cohort included 100 women with HCM with one or more live births, for a total of 199 live births. Morbidity related to HCM during pregnancy was investigated in 40 women evaluated within five years of their pregnancy.

RESULTS: Two pregnancy-related deaths occurred, both in patients at a particularly high risk. The maternal mortality rate was 10 per 1,000 live births (95% confidence interval [CI] 1.1 to 36.2/1,000) and was in excess of the expected mortality in the general Italian population (relative risk 17.1, 95% CI 2.0 to 61.8). In the 40 patients evaluated within close proximity of their pregnancy, 1 (4%) of the 28 who were previously asymptomatic and 5 (42%) of the 12 with symptoms progressed to functional class III or IV during pregnancy (p < 0.01). One patient had atrial fibrillation and one had syncope, both of whom had already experienced similar and recurrent events before their pregnancy.

CONCLUSIONS: Maternal mortality is increased in patients with HCM compared with the general population. However, absolute maternal mortality is low and appears to be principally confined to women at a particularly high risk. In the presence of a favorable clinical profile, the progression of symptoms, atrial fibrillation, and syncope are also uncommon during pregnancy.

Abbreviations and Acronyms   CI   confidence interval   HCM   hypertrophic cardiomyopathy   LV   left ventricle or ventricular

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