Two Cases Of Cardiac Sarcoidosis In Pregnant Women With .

96CARDIOVASCULAR JOURNAL OF AFRICA Volume 26, No 2, March/April 2015AFRICATwo cases of cardiac sarcoidosis in pregnant womenwith supraventricular arrhythmiaEbru Ertekin, Sulaiman Moosa, Jolien W Roos-Hesselink, Karen SliwaAbstractWe present two cases of cardiac sarcoidosis whose first presentation was in pregnancy. All findings confirmed the diagnosis of sarcoidosis with cardiac involvement in both patients.The first patient, a 37-year-old, presented with dizzinessand atrial fibrillation at 16 weeks’ gestation. Echocardiography revealed thickened interventricular septum with a speckled pattern. Cardiac MRI after delivery showed myocardialoedema/inflammation corresponding with the same regionswith early enhancement and epicardial delayed enhancementin the basal to mid-inferoseptal and basal anterior left ventricular myocardial segments. Transbronchial biopsy revealedhistology of scanty fragments of inflamed bronchial mucosa.The second patient, a 31-year-old, was 17 weeks pregnant when she presented with daily palpitations and shortness of breath. She had prolonged episodes of supraventricular tachycardia. Echocardiography revealed a speckledseptal and right ventricular wall pattern. Cardiac MRI afterdelivery showed basal and mid-ventricular mesocardial andepicardial enhancement, most compatible with sarcoidosis.Keywords: cardiac sarcoidosis, pregnancy, arrhythmiaSubmitted 2/10/14, accepted 3/2/15Cardiovasc J Afr 2015; 26: 96–100www.cvja.co.zaDOI: 10.5830/CVJA-2015-022Case 1: patient with atrial fibrillationA 37-year-old primigravida presented to our cardiac clinicat 16 weeks’ gestation with a history of dizziness and atrialfibrillation (AF) without overt heart disease. She took no regularmedication, did not smoke or drink alcohol, and had no diabetesmellitus or hypertension. Her medical history included AFdiagnosed six years previously, and polycystic ovarian syndrome.There was no cardiac disease or sudden death in her family.Physical examination revealed an irregular tachycardia of130 beats per minute (bpm), blood pressure of 124/72 mmHgDepartment of Cardiology, Erasmus University MedicalCenter, Rotterdam, The NetherlandsEbru Ertekin, BScJolien W Roos-Hesselink, MD, PhDDepartment of Radiology, 2 Military Hospital, Cape Town,South Africaand no murmur. The electrocardiogram showed ventricularectopics, intermittent AF and irregular P-wave morphology (Fig.1). Echocardiography revealed a normal heart with an ejectionfraction of 60%. She was prescribed atenolol 25 mg daily andaspirin 75 mg daily due to the risk of a cerebrovascular accident.Echocardiography was repeated one month later and showeda thickened speckled septum and posterior wall with good leftventricular function (left ventricular ejection fraction 61%, leftatrial diameter 34 mm, aortic diameter 28 mm, left ventricularend-diastolic diameter 53 mm). These findings were suspiciousfor an infiltrative disease, and cardiac sarcoidosis was consideredto be the most probable diagnosis. The obstetric–cardiac teammade a decision at that time to perform a full work-up forcardiac sarcoidosis post delivery.She delivered under spinal and general anaesthetia at 38weeks of gestation by caesarean section due to breech position.She gave birth to a healthy female baby of 2 780 g. On dischargeshe was prescribed aspirin 80 mg, eltroxin 100 μg and atenolol50 mg. Three months post partum she had mild to moderateshortness of breath on effort, her blood pressure was 110/78mmHg and her heart rate was 70–80 bpm while she was in AF.MRI was performed four months after delivery anddemonstrated the following findings: preserved biventricularsystolic function and volumes, myocardial oedema orinflammation corresponding to the same regions with earlyenhancement and epicardial delayed enhancement in the basal tomid-inferoseptal and basal anterior left ventricular myocardialsegments (Fig. 2). With these MRI findings, cardiac sarcoidosiswas considered to be the most probable diagnosis. However,myocarditis was a less likely differential consideration.CT chest demonstrated multiple bilateral pulmonary nodulespredominantly in a perilymphatic distribution. Transbronchialbiopsy confirmed the diagnosis of sarcoidosis. Microscopicexamination showed scanty fragments of inflamed bronchialmucosa. Granulomas and viral inclusions were not seen.Additionally, serum angiotensin converting enzyme level was23.8 U/l (normal range: 9–67 U/l).Steroid therapy was started after the diagnosis of sarcoidosisby transbronchial biopsy. At the follow-up visit 10 months afterdelivery, her symptoms had improved and she was on atenolol25 mg daily, prednisone 40 mg daily, with calcium supplementation,vitamin D, isoniazid 200 mg daily (antibiotics) and eltroxin100 mcg daily.All findings confirmed the diagnosis of sarcoidosis. As therewas cardiac involvement, steroid therapy was prescribed. Steroidtherapy was successful and improved her symptoms.Sulaiman Moosa, MDHatter Institute for Cardiovascular Research in Africa,MRC Inter Cape Heart Group, Department of Medicine,University of Cape Town, South AfricaKaren Sliwa, MD, PhD, karen.sliwa-hahnle@uct.ac.zaCase 2: patient with supraventriculartachycardiaThe second patient we saw was a 31-year-old woman who was17 weeks pregnant with her third pregnancy. She had prolonged

AFRICACARDIOVASCULAR JOURNAL OF AFRICA Volume 26, No 2, March/April 201597Fig. 1. E CG of case 1, performed when the patient was 16 weeks pregnant, showing intermittent atrial fibrillation.episodes of supraventricular tachycardia of unknown origin withalmost daily palpitations on minimal effort. At that time she wasdiagnosed with NYHA functional class II failure off medication(she was not on any medication at presentation). Her motherhad sarcoidosis. Her obstetric history included two threatenedmiscarriages and two viable births, both vaginal delivery.Physical examination was normal and her heart rate was 78bpm. Holter monitoring did not identify ventricular tachycardia.Echocardiography revealed a speckled pattern at the basalsegment of the septum and right ventricular wall, suspiciousfor an infiltrative disease. Left ventricular function was good(LVEF 53%) and no valve dysfunction was observed. In viewof the clinical history, those features were highly suggestive ofsarcoidosis with cardiac involvement. Verapamil 40 mg daily wasstarted and MRI investigation was booked after pregnancy forfurther investigation.One month later she presented with increased palpitations onverapamil. She had previously responded to verapamil but didnot respond on atenolol or propanolol. The palpitations startedwithout any obvious trigger and lasted one hour. Haemoglobinwas 11.7 g/dl (normal range, non-pregnant woman: 12.0–15.5g/dl) and thyroid stimulating hormone was 1.29 mU/l (normalrange: 0.34–4.25 mU/l). She had normal electrocardiography.Verapamil dosage was increased to 80 mg because of theincreased complaints of palpitations.At 30 weeks of pregnancy, she presented at the clinic withdaily palpitations and shortness of breath. The palpitationsdid not improve on verapamil. Her blood pressure was 108/58mmHg, she had a regular heart rate of 104 bpm (sinus rhythm)and was not in congestive heart failure. At that time verapamilwas stopped due to its possible side effects on the foetus.She delivered at 36 weeks of gestation by emergency caesareansection due to foetal distress. She gave birth to a healthy malebaby of 2 760 g. Echocardiography was repeated three monthspostpartum and demonstrated a speckled pattern, diastolic leftventricular dysfunction with right ventricular function of 50%and left ventricular function of 68%.MRI was performed four months after delivery and showeda mildly enlarged left ventricle, reduced LVEF ( 50%) but noregional wall motion abnormality. The delayed gadoliniumsequences showed basal and mid-ventricular mesocardial andepicardial enhancement (Fig. 3). The radiologist concluded thatthese features could be consistent with sarcoid-related myocardialscarring. The patient declined to have a transbronchial biopsy.After starting steroid therapy, there was a marked improvementin her symptoms, including shortness of breath. The cardiac MRIwas repeated and showed a marked increase in LVEF (from 50%before starting steriod therapy to 70% after starting the therapy).DiscussionOur cases demonstrate that cardiac sarcoidosis may manifest forthe first time during pregnancy. Reports on cardiac sarcoidosisrelated to pregnancy are rare.1,2 Cardiac sarcoidosis, a potentially

98CARDIOVASCULAR JOURNAL OF AFRICA Volume 26, No 2, March/April 2015ABCDAFRICAFig. 2. C ardiac MRI images of case 1. A: basal short-axis T2W slice, demonstrating patchy high signal of the inferoseptal myocardium (white arrow). B: basal short-axis T1W early post-gadolinium image, revealing inhomogeneous myocardial enhancement (white arrow). C and D: basal (C) and mid-ventricular (D) short-axis phase-sensitive inversion recovery (PSIR) delayedenhancement images, demonstrating anterior basal (black arrow in C) as well as inferoseptal epicardial enhancement (whitearrows in C and D).life-threatening condition, is probably an underdiagnosed disease,as cardiac involvement is clinically apparent in only about 5% ofall patients, while cardiac granulomas are found in about 25% ofpatients with sarcoidosis who are examined at autopsy.3 Cardiacsarcoidosis may occur without apparent disease in other organs.It has been reported to occur most frequently in middle-agedor older women in Japan, although its incidence in Westerncountries was shown to have no gender difference and to behigher in the younger generation.4,5Sarcoidosis in pregnancyThe incidence of sarcoidosis in pregnancy is estimated to be0.02 to 0.05%.6 Maternal sarcoid symptoms may improve duringpregnancy but may be more severe after delivery. The changesin the severity of sarcoidosis during pregnancy are possibly dueto increased serum steroid levels, which rapidly decrease afterdelivery.7 On the other hand, sarcoidosis can progress duringpregnancy, or as our two cases show, manifest for the firsttime during pregnancy. Sudden death secondary to ventricular