A preterm male baby (gestational age of 35+ 6 weeks, body weight 2.74 kg) was transferred to the neonatal intensive care unit (NICU) of the Army Medical Center in May 2020 for slight cyanosis around the lips and nose. The baby was delivered by C-section for intrauterine stress, and the second birth of a mother with psoriasis and severe intrahepatic cholestasis. The mother had regular antenatal care including the fetal heart monitoring, but without treatment for her ten-year psoriasis. Meanwhile, the fetal bradycardia was not detected during the antenatal follow-up. Prior to delivery, The mother’s serum antibodies were ANA (1:1000), SSA (+++), SSB (+++), and Ro-52 (+++). The baby’s myocardial enzyme spectrum after admission indicated an increasing in creatine kinase-myocardial band isoenzyme (CK-MB). Electrocardiogram (ECG) monitoring showed a sudden onset of heart rate fall, P (142 bpm) and QRS (78 bpm) were isolated (IIIAVB), ventricular escape beat, incomplete right bundle branch block, with ST-T change at 3 days after birth (Fig. 1A). Addtionally, deoxygenation [oxygen saturation (SpO2) < 80%] with severe myocardial injury and acute heart failure [brain natriuretic peptide (BNP) > 5000 pg/mL] were observed immediately and persisted. Physical examination showed a bluish-purple coloration around the lips, a red rash on the anterior chest, irregular heartbeat with weak and uneven heart sounds, and blood pressure of 76/44 mmHg. Ultrasound showed generalized cardiac enlargement, severe mitral valve regurgitation, severe tricuspid regurgitation, pulmonary artery hypertension, bradycardia, and segregation of atrial septal anomalies. ECG showed sinus rhythm, ectopic rhythm,and IIIAVB. (Fig. 1B). Chest X-ray showed generalized cardiac enlargement. The immunofluorescence density of SSA, SSB, and RO-52 antibodies was strongly positive, and ANA was 1:320. Brain magnetic resonance imaging (MRI) showed white matter injury (WMI) under the right side of the parietal lobe, with a significant elevation of tumor necrosis factor (TNF)-α and interleukin (IL)-6. CHB-NL, heart failure, and dilated cardiomyopathy was diagnosed.

ECG records before and after plasmapheresis. A (3 days after birth) the first EEG on the beginning of AVB. P(142 bpm) and QRS (78 bpm) were isolated, ventricular escape beat, incomplete right bundle branch block, with ST-T change. B (5 days after birth) Sinus rhythm, ectopic rhythm and IIIAVB. C (7 days after birth) Sinus rhythm, frequent Ventricular premature beat. D (9 days after birth) Sinus rhythm and occasional atrial premature beats. E Sinus rhythm

The antiarrhythmic drugs isoproterenol and adrenaline used to increase the ventricular rate and enhance cardiac output in the early stage (< 24 h) soon became ineffective, and intractable heart failure led to agonal stage signs 24 h after the break. Transcutaneous pacing was performed using a defibrillator (M series, ZOLL Medical Corporation, Chelmsford, MA, USA) with the pace function module with a pace current of 30 mA and pace heart rate (HR) of 120 bpm. Plasmapheresis and hormone shock therapy were performed. After the first plasmapheresis session, ECG monitoring showed sinus rhythm, frequent Ventricular premature beat (Fig. 1C). The second and third plasmapheresis sessions reversed the CHB back to occasional atrial premature beats (Fig. 1D). When a new complete right bundle branch block was detected, two partial plasmapheresis sessions were performed, and the HR returned to alternate incomplete right bundle branch block or normal. After 1 month of birth, the patient was discharged from the hospital with an HR of 130–148 bpm (Fig. 1E) and the antibody titers were SSA (+++), SSB (+), and Ro-52 (+++) (Table 1). At 1-year follow-up after birth, growth and development were normal. SSA, SSB, and Ro-52 were negative. MRI showed no significant changes in the softening lesion in the right parietal lobe. The baby had grown up healthy to 1.5 years, with a well-developed cardiac structure and normal neurodevelopment scores.