Volume 4, Number 12;  July 21, 2005

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Patient:

Session Handout:

Clinical Question: 

What are factors leading to progressive disease or mortality in Eisenmenger syndrome generally, and what is the outlook for a female patient with Eisenmenger's who might become pregnant?

Readings:

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Link Directly to Fulltext Article at Science Direct

Unique Identifier [PMID]: 10400015

Authors: Niwa K. Perloff JK. Kaplan S. Child JS. Miner PD.

Institution: Department of Medicine, The Ahmanson/UCLA Adult Congenital Heart Disease Center, University of California, Los Angeles, USA.

Title: Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart.

Source: Journal of the American College of Cardiology. 34(1):223-32, 1999 Jul.

Abstract: OBJECTIVES: Morbidity and mortality patterns were characterized in adults with the Eisenmenger syndrome when two ventricles with a ventricular septal defect (VSD) joined two great arteries or one great artery, or when one ventricle joined two great arteries. BACKGROUND: Although afterload in these disorders differs, clinical differences have not been defined. METHODS: Seventy-seven patients were studied. Group A comprised 47 patients with VSD, aged 23 to 69 years (mean 39.5+/-10.2), follow-up 5 to 18 years (mean 7.2+/-4.9); group B, 14 patients with truncus arteriosus, aged 27 to 50 years (mean 33.7+/-7.3), follow-up 6 to 18 years (mean 7.7+/-5.1), and group C, 16 patients with univentricular heart, aged 18 to 44 years (mean 30.6+/-8.4), follow-up 5 to 15 years (mean 4.4+/-4.2). Echocardiography established the diagnoses and anatomic and hemodynamic features. Data were compiled on tachyarrhythmias, pregnancy, infective endocarditis, noncardiac surgery and the multisystem disorders of cyanotic adults. RESULTS: Thirty-five percent of the patients died. Sixty-three percent of deaths were sudden, and resulted from intrapulmonary hemorrhage, rupture of either the pulmonary trunk, ascending aorta or a bronchial artery, or vasospastic cerebral infarction, or the cause was unestablished. There were no documented tachyarrhythmic sudden deaths. CONCLUSIONS: Medical management of coexisting cardiac disease, multisystem systemic disorders, noncardiac surgery and pregnancy has reduced morbidity. Increased longevity exposed patients to proximal pulmonary arterial aneurysms, thromboses and calcification; to truncal valve stenosis and regurgitation; to semilunar and atrioventricular valve regurgitation, and to major risks of nontachyarrhythmic sudden death.

Publication Type: Journal Article.

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Unique Identifier [PMID]: 9886728

Authors: Daliento L. Somerville J. Presbitero P. Menti L. Brach-Prever S. Rizzoli G. Stone S.

Institution: Division of Cardiology, University of Padua, Italy.

Title: Eisenmenger syndrome. Factors relating to deterioration and death.

Source: European Heart Journal. 19(12):1845-55, 1998 Dec.

Abstract: AIMS: To assess the natural history, risk factors for death and deterioration of patients with Eisenmenger Syndrome. METHODS: The clinical course of 188 patients from three different cardiac centres specialized in adult congenital heart defects, followed for a median period of 31 years, was retrospectively analysed. According to the diagnosis, 64 males and 124 females, mean age at last follow-up 33.02+/-12.8 years, were divided into two groups: simple (128) and complex (60) congenital heart disease. Events analysed were: time variation of Ability Index, pulmonary and cerebral complications, non-cardiac surgery, urate metabolism and renal function, arrhythmias, pregnancy and contraception, medical therapy and transplantation, death from all causes and sudden death. RESULTS: Eighty-four percent of the overall population showed a satisfactory Ability Index (1 and 2) at the first attendance (median age 25 years). Patients with complex heart disease and Eisenmenger reaction had earlier clinical deterioration (18.6+/-11.3 vs 26.7+/-12.2) and shorter survival (25.8+/-7.9 vs 32.5+/-14.6 years). Thirty-eight (20.2%) patients had at least one episode of haemoptysis between 20 and 40 years of age but it did not modify overall survival; 25 (13.2%) had pulmonary thromboembolism at a mean age of 35.2+/-13.4 years. Fifteen (7.9%) had a stroke and 7 (3.7%) a cerebral abscess at a mean age of 31.4+/-15.7 and 24.1+/-4.9 years, respectively. Cerebral complications influenced the quality of life but did not modify survival. Patients who had venesection showed a 2.04 times greater hazard ratio for haemoptysis. Venesection did not reduce cerebral complications and in 20% caused anaemia and iron deficiency. Other non-cardiac surgery with general anaesthesia carried risks (23.5% of deaths). Significant maternal mortality (27%) in relation to pregnancy occurred with constant deterioration in physical status, high incidence of spontaneous abortions (35.8%) and cardiac abnormalities in offspring (20%). Sixty-one patients died during follow-up, mainly by sudden death (29.5%), heart failure (22.9%) and from haemoptysis (11.4%). Eight patients had heart and lung transplantation and five died 1 week to 4 years after transplant. Deterioration in Ability Index (worsening symptoms), age, complex defects, blood creatinine level, right ventricular dysfunction and non-cardiac surgery were variables which affected the prognosis adversely with uni- and multivariate analysis. CONCLUSIONS: patients with Eisenmenger syndrome can survive to the seventh decade with informed medical care and protection from special risks

Publication Type: Journal Article. Multicenter Study.

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Unique Identifier [PMID]: 9746388

Authors: Yentis SM. Steer PJ. Plaat F.

Institution: Chelsea and Westminster Hospital, London.

Title: Eisenmenger's syndrome in pregnancy: maternal and fetal mortality in the 1990s.

Source: British Journal of Obstetrics & Gynaecology. 105(8):921-2, 1998 Aug.

Abstract: To determine maternal and fetal mortality associated with Eisenmenger's syndrome in the UK, a postal questionnaire was sent to 225 NHS obstetric units with neonatal intensive care units, requesting information about maternal and fetal outcome in cases of Eisenmenger's Syndrome between 1991 and 1995. Fifteen cases were identified. The maternal mortality was 40% and fetal loss 8%. Only 15% of infants were born at term. Maternal mortality associated with Eisenmenger's syndrome remains as high as it has been for the past 50 years. Pooling of national data on rare medical conditions in pregnancy is required to aid management of individual cases.

Publication Type: Journal Article.

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Link Directly to Fulltext Article at Science Direct

Unique Identifier [PMID]: 9626847

Authors: Weiss BM. Zemp L. Seifert B. Hess OM.

Institution: Department of Anesthesiology, University Hospital, Zurich, Switzerland.

Title: Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. [Review] [109 refs]

Source: Journal of the American College of Cardiology. 31(7):1650-7, 1998 Jun.

Abstract: OBJECTIVES: Published reports were reviewed to evaluate the characteristics of peripartal management and the late pregnancy outcome in women with pulmonary vascular disease (PVD). BACKGROUND: Pulmonary hypertension poses one of the highest risks for maternal mortality, but actual data on the maternal and neonatal prognosis in this group are lacking. METHODS: Reports published from 1978 through 1996 of Eisenmenger's syndrome (n = 73), primary pulmonary hypertension (PPH) (n = 27) and secondary vascular pulmonary hypertension (SVPH) (n = 25) complicating late pregnancy were included and analyzed using logistic regression analysis. RESULTS: Maternal mortality was 36% in Eisenmenger's syndrome, 30% in PPH and 56% (p < 0.08 vs. other two groups) in SVPH. Except for three prepartal deaths due to Eisenmenger's syndrome, all fatalities occurred within 35 days after delivery. Neonatal survival ranging from 87% to 89% was similar in the three groups. Previous pregnancies, timing of the diagnosis and hospital admission, operative delivery and diastolic pulmonary artery pressure were significant univariate (p < 0.05) maternal risk factors. Late diagnosis (p = 0.002, odds ratio 5.4) and late hospital admission (p = 0.01, odds ratio 1.1 per week of pregnancy) were independent predictive risk factors of maternal mortality. CONCLUSIONS: In the last two decades maternal mortality was comparable in patients with Eisenmenger's syndrome and PPH; however, it was relevantly higher in SVPH. Maternal prognosis depends on the early diagnosis of PVD, early hospital admission, individually tailored treatment during pregnancy and medical therapy and care focused on the postpartal period. [References: 109]

Publication Type: Journal Article. Review. Review, Tutorial.

Resident Report / Department of Medicine & Grady Branch Library

Emory University School of Medicine

2005 Edition

Participating Faculty:  Carlos Del Rio MD  / Joyce Doyle MD / Lorenzo Difrancesco MD / Erich Folch MD / Alicia Hidron  MD  

Contact: Karl Woodworth