Reported cardiac phenotypes in hereditary hemorrhagic telangiectasia emphasize burdens from arrhythmias, anemia and its treatments, but suggest reduced rates of myocardial infarction.

INTRODUCTION: Cardiac phenotypes should be pronounced in hereditary hemorrhagic telangiectasia (HHT) due to frequent systemic arteriovenous malformations (AVMs), iron deficiency anemia, hypoxemia, hyperdynamic circulations, venous thromboemboli, and paradoxical emboli through pulmonary AVMs. METHODS/RESULTS: In an international survey, 1025 respondents (median age 55years) met HHT diagnostic criteria: 942 (91.9%) reported nosebleeds, 452 (44.1%) at least daily. AVMs were commonly reported in pulmonary (544, 53%), hepatic (194, 18.9%) and/or cerebral (92, 9.0%) circulations. 770/1025 (75%) had used iron tablets, 256 (25.0%) intravenous iron, and 374 (36.5%) received blood transfusions. Arrhythmias were reported by 113/1025 (11%, including 44 (4.3%) with atrial fibrillation), angina by 36 (3.5%), and cardiac failure by 26 (2.5%). In multivariate logistic regression, these phenotypes were associated with hepatic AVMs/pulmonary hypertension (relatively interchangeable variables), blood transfusions, and intravenous iron. Cardiac insufficiency/failure often provokes intensive anemia treatments, but associations with arrhythmias, particularly with a greater transfusion burden, were less easy to explain. Myocardial infarction (23/1025; 2.2%), and abnormal coronary angiogram (≤31/76, ≤54%) rates appeared low. Provocative preliminary data were obtained including HHT-affected respondents' parents and grandparents in whom HHT could be confidently assigned, or excluded based on autosomal dominant inheritance patterns: in crude and survival analyses, myocardial infarctions were reported less frequently for individuals with HHT, particularly for males (p=0.001). CONCLUSION: Arrhythmias are the most common cardiac phenotype in HHT, and likely to be aggravated by iron deficiency anemia, its treatments, and/or high output states due to AVMs. Myocardial infarction rates may be reduced in this apparently high risk population.

Predictors of survival and the role of gender in postoperative myocardial infarction.

PURPOSE: To identify risk factors for mortality after postoperative myocardial infarction. METHOD: Retrospective study of 266 patients. RESULTS: The crude in-hospital mortality rate was 25%. This was more than twice as high as the mortality rate in patients admitted from home with an acute myocardial infarction. Women with postoperative infarction were the same age as men, but had a lower Acute Physiology and Chronic Health Evaluation (APACHE) II score prior to infarction (P = 0.03) and a higher crude mortality rate. Multivariate analysis showed that female gender (relative risk 2.2, 95% confidence limits 1.2 to 4.2), current cigarette smoking (relative risk 2.3 [1.2 to 4.7]), a history of congestive heart failure (relative risk 2.1 [1.04 to 4.1], resuscitation status (relative risk 8.1 [2.0 to 32.9]), and high preoperative APACHE II score were significant independent predictors of in-hospital mortality. CONCLUSION: Postoperative myocardial infarction is one of the most serious events a patient can experience. Women and current smokers are at especially high risk for mortality after postoperative myocardial infarction.