Pulmonary hypertension associated with auto-immune complete heart block in neonates: Unrecognized manifestation of neonatal lupus

Auto-immune complete heart block (CHB) is a well-defined consequence of antenatal exposure to maternal anti-SSA/Ro and/or anti-SSB/La antibodies. After birth, the persistence of maternal anti-SSA and/or anti-SSB antibodies passively transferred to the foetus can cause skin rash, vascular, hepatic or neurologic manifestations. Pulmonary involvement is infrequent in a such condition in neonates while pulmonary hypertension (PH) is a common complication of systemic lupus erythematosus (SLE) in adulthood. We report a series of 4 neonates referred to our institution for prenatal diagnosis of auto-immune CHB who developed pulmonary hypertension after birth. Two patients were paced at the time of PH diagnosis and 2 patients weren’t. PH was suspected by transthoracic echocardiography after a PH-free interval ranging from 10 days to 3 months. PH was confirmed by right heart cath (RHC) in all, all had a lung CT-pulmonary angiography and one patient had a pulmonary biopsy at the time of pacemaker implantation. At the RHC all the patients presented a pre-capillary PH (PAPm > 25 mmHg, PWAP