Management of a rare case of arrhythmogenic right ventricular dysplasia in pregnancy: a case report

Abstract

IntroductionArrhythmogenic right ventricular dysplasia is a heritable disease of the heart muscle characterized by fibrofatty degeneration of cardiomyocytes. Patients present with ventricular arrhythmias or congestive heart failure, and sometimes sudden cardiac death occurs. Prenatal diagnosis has become possible with the detection of mutations, but, to the best of our knowledge, no case of prenatal diagnosis has been reported previously. There is little information about the management of arrhythmogenic right ventricular dysplasia in pregnancy, and the preferred mode of delivery is not certain; therefore, we present the case of a patient with arrhythmogenic right ventricular dysplasia and discuss the prenatal diagnosis, patient management and prognosis in pregnancy.Case presentationA 26-year-old Caucasian woman who presented to our hospital with heart palpitations was diagnosed with arrhythmogenic right ventricular dysplasia, and, after three years of follow up with anti-arrhythmic drugs, she wanted to conceive. During pregnancy, she ceased taking her medication. She tolerated pregnancy very well but her cardiac symptoms recurred after her 30th week of pregnancy. She delivered a baby via cesarean section under general anesthesia in her 38th week of pregnancy. She was discharged without any medications and continued lactation for six months.ConclusionPatients with mild to moderate arrhythmogenic right ventricular dysplasia tolerate pregnancy and breastfeeding very well, but patients with end-stage arrhythmogenic right ventricular dysplasia should be discouraged from conception.

Arrhythmogenic Right Ventricular Cardiomyopathy in Pregnancy

“…In a case reported by Iriyama, et al, 18) the propranolol treatment that the patient had been taking was not given during the pregnancy on her own request and VT occurred at the 34 th week. Similarly in the case of Güdücü, et al, 17) the patient taking regular metoprolol and propafenone treatment for 3 years with the diagnosis of ARVC refused to use the drugs on her own request and she became symptomatic at the 32 nd week. In her Holter ECG at the 32 nd week, frequent PVC attacks were detected.…”

“…[14][15][16][17][18] Therefore, it is difficult to assess the risks of pregnancy and delivery in patients with ARVC. Furthermore, sustained VT developed during the pregnancy in only one of these cases.…”

“…The remaining two cases with only isolated PVC or no arrhythmias underwent spontaneous vaginal delivery. Iriyama, et al 17) preferred to perform elective caesarean section due to a history of major arrhythmias. Our patient delivered by caesarean section due to fetal indication (oligohydramnios).…”

Successful Obstetric Management of Arrhythmogenic Right Ventricular Cardiomyopathy

“…A case reported by Iriyama et al [18] documented that discontinuation of antiarrhythmic therapy during pregnancy may result in the development of major ventricular arrhythmias for example. On the other hand, in a case report, Güdücü et al [19] showed that discontinuation of β-blocker therapy may not compromise a successful pregnancy, even in a patient advised to take β-blocking agents to prevent arrhythmias; the patient experienced heart palpitations, and ECG Holter monitoring at the 32nd week of gestation showed >16,000 ventricular extrasystoles.…”

“…We proposed genetic counseling about the risk of inheritance of cardiac defects for her child because genetic analysis can avoid the lifelong cardiological follow-up in the child if the mutation is not detectable [19]. This analysis cannot predict with certainty the clinical phenotype and risk stratification, because the final phenotype is also determined by incomplete penetrance and pleiotropic effects, and may vary significantly.…”