Abstract
Aortic dissection in pregnancy is a lethal cardiovascular complication in women with Marfan syndrome. The course of the treatment would be decided upon by the size of aortic root dilation. We report a case where the unawareness of the diagnosis of Marfan syndrome antenatally led to disastrous post partum consequences even after an uneventful childbirth. DOI: http://dx.doi.org/10.4038/sljog.v34i2.4829 Sri Lanka Journal of Obstetrics and Gynaecology 2012; 34 : 55-57
Highlights
Marfan syndrome is one of commoner autosomal dominant single gene malformation syndromes that affect both sexes in any ethnic group
Closer scrutiny revealed that she had developed a spontaneous pneumothorax at the age of 22 years which was not further investigated
Vigilant attention at the time of the spontaneous pneumothorax and during her pregnancies coupled with family history should have helped to make the diagnosis in this patient at an earlier point of time
Summary
Marfan syndrome is one of commoner autosomal dominant single gene malformation syndromes that affect both sexes in any ethnic group. It is a multisystem disorder resulting in diminished strength of connective tissues mostly affecting cardiovascular, ocular and skeletal systems. On the thirteenth postpartum day she was admitted to hospital with worsening shortness of breath On admission she was found to have tachycardia, low blood pressure, and evidence of pulmonary oedema. Electrocardiogram showed sinus tachycardia and ST depressions change in lateral chest leads (V3-V6) chest leads. She died six hours after admission despite attempted resuscitation and treatment. Closer scrutiny revealed that she had developed a spontaneous pneumothorax at the age of 22 years which was not further investigated
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