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A young lady with palpitations.

A 31 years old female, was first seen because of occasional complaints of premature beats. She had a normal physical examination with only slightly elevated blood pressure of 145/90. An echo showed a left ventricular chamber measuring 55mm end-diastolic and 45mm end-systolic with normal systolic and diastolic function.   NTproBNP was 302 ng/L (slightly elevated). Her ECG was as shown in figure 1.

On Holter monitoring she showed 80 VPBs of varying morphologies. She reports no cardiac disease in her family, denies any other cardiac complaint like chest pain or dyspnea on exertion. Her exercise test is unremarkable, she attains a total of 11 METS without arrhythmias or ST-T abnormalities.
She is scheduled for a routine check-up but only shows up more than 2 years later. A repeated echocardiogram now shows end diastolic diameter of 57 mm, end-systolic diameter 49mm, EF on biplane is 44%. NTproBNP has risen to 1184 ng/L.
On repeat inquiry of the family she tells us that she has a nephew who is in a wheel chair.  

Questions to be answered:

  • Question 1 What is your diagnosis?

Answer: female carrier of Duchenne mutation.
She is installed on a beta-blocker and an ACE inhibitor. After a year she asks our advice since she wants to become pregnant.

  • Question 2 What is your advice?

Answer: pregnancy is not forbidden given the ejection fraction but needs close monitoring, at least in the period around the first twenty weeks.
Betablocker should be continued, ACE inhibitor should be stopped when setting up the pregnancy.
After the advice she becomes pregnant and cardiac investigations are repeated at 22 weeks of pregnancy. Systolic function has declined to an EF of 28%, LVIDD 59mm. On Holter monitoring she has 4 triplets , polymorphic of 200 bpm.

  • Question 3 Should pregnancy be terminated because of worsening cardiomyopathy?

Answer: no: according to ESC guidelines EF< 40% is higher risk and needs close monitoring, only with EF <20%  termination of pregnancy is considered. A multidisciplinary guidance of rest of pregnancy and delivery is organized.
She delivers a healthy baby girl after 39 weeks of pregnancy, but has a poor left ventricular function by that time with an EF of 20%. After delivery cardiac function restores a little bit but does stay around  EF 35%, and she receives an ICD.

Notes to editor

Presented by Ygal Pinto MD, FESC
Department of Cardiology (Heart Failure Research Center), Academic Medical Center, Amsterdam, The Netherlands.
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.