A 32 year old woman presented at 29 weeks gestation with reduced fetal movements and abdominal pain to her local hospital. An ultrasound scan revealed gross fetal hydrops secondary to congenital complete heart block (cCHB) with a ventricular rate of 40 bpm. The baby was transferred in-utero to the regional fetal medicine centre whereby the multidisciplinary team opted for immediate caesarean section for the best possible chance of survival for the baby.
Following delivery the baby was intubated, ventilated and started on isoprenaline and dobutamine infusions. The heart rate remained at 40–50 bpm so the baby was urgently transferred to the tertiary cardiac centre for drainage of ascites and pleural effusions and insertion of emergency epicardial pacing wires. Echocardiography confirmed a structurally normal heart. On day 13 a permanent pacemaker was inserted with a VVI pacing mode set at 130 ppm. She was extubated day 16 weighing just under 1.2 kilograms with her hydrops resolved. The mother was found to be positive for anti-Ro (SSA) antibodies, despite being asymptomatic from rheumatological disease. At 8 months of age the child is asymptomatic, thriving and developing appropriately.
Premature hydropic babies with cCHB have an extremely poor prognosis with a high mortality. There are only a few reported cases in the literature with good outcomes of infants with severe hydrops of this gestation and weight surviving and having successful pacemaker insertion.
This case highlights its success following initial medical stabilisation in a tertiary neonatal unit with a close link to tertiary cardiac care.
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