Management of Friedreich Ataxia-Associated Cardiomyopathy in Pregnancy: A Review of the Literature.

A major manifestation of Friedreich ataxia (FRDA) is cardiomyopathy, caused by mitochondrial proliferation in myocytes. Because the lifespan for patients with FRDA improves with better treatment modalities, more patients are becoming pregnant, meaning that more medical providers must know how to care for this population. This report provides a review of the literature on multidisciplinary management of pregnant patients with FRDA and cardiomyopathy from preconception through lactation. A cardio-obstetrics team, including cardiology, anesthesiology, and obstetrics, should be involved for this entire period. All patients should be counseled on pregnancy risk using elements of existing stratification systems, and contraception should be discussed, highlighting the safety of intrauterine devices. Electrocardiogram should be obtained at baseline and each trimester, looking for atrial arrhythmias and ST-segment changes, as should transthoracic echocardiogram, with a focus on left ventricular ejection fraction-which is typically normal in FRDA cardiomyopathy-and relative wall thickness and global longitudinal strain-which tend to decrease as cardiomyopathy progresses. Brain natriuretic peptide is also a helpful marker to detect adverse events. If heart failure develops, it should be treated like any other etiology of heart failure during pregnancy. Atrial arrhythmias should be treated with ß blockers or electrical cardioversion and anticoagulation, as necessary. Most patients with FRDA can deliver vaginally, and neuraxial analgesia is recommended during labor because of the risks associated with general anesthesia. Breastfeeding is encouraged, even for those taking cardiac medications.

Successful Pregnancy After Cardiac Arrest in a Woman With Severe Coronary Vasospasm.

A 37-year-old gravida 5, para 3 woman presented with an unplanned pregnancy 6 weeks after experiencing a cardiac arrest caused by ventricular fibrillation from coronary vasospasm. She opted to continue the pregnancy with medical management despite ongoing chest pain and delivered a healthy female infant via vaginal delivery at 37 weeks.

Association between Abnormal Echocardiography and Adverse Obstetric Outcomes in Low-Risk Pregnant Women.

Maternal mortality in the United States is a public health crisis of preventable deaths among young women. The role of echocardiography in the evaluation of pregnant women with cardiovascular symptoms or risk factors without known heart disease is unclear. We retrospectively examined the clinical characteristics of consecutive pregnant patients without established heart disease who underwent echocardiography and evaluated associations between abnormal exam findings and obstetric outcomes. Among low-risk women undergoing echocardiography during pregnancy, older age, higher parity and a history of chronic hypertension are associated with a higher likelihood of echocardiographic abnormalities, which in turn are associated with a higher likelihood of adverse obstetric outcomes including caesarean section and preterm delivery.

Pheochromocytoma, Fulminant Heart Failure, and a Phenylephrine Challenge. the Perioperative Management of Adrenalectomy in a Jehovah's Witness Patient: a Case Report.

Perioperative management of pheochromocytoma in the setting of catecholamine-induced heart failure requires careful consideration of hemodynamic optimization and possible mechanical circulatory support. A Jehovah's Witness patient with catecholamine-induced acutely decompensated heart failure required dependable afterload reduction for a cardio-protective strategy. This was emphasized due to the relative contraindication to perioperative anticoagulation required for mechanical circulatory support. A phenylephrine challenge clearly demonstrated adequate alpha blockade after only 24 hours of phenoxybenzamine treatment. This resulted in advancement of the surgery date. This case also highlights management of beta blockade, volume and salt loading, autologous blood transfusion, and profound post-operative vasoplegia in the setting of cardiogenic shock. Careful attention to hemodynamic optimization and cardio-protective strategies ultimately resulted in positive outcome for this challenging clinical scenario.