Pulmonary hypertension associated with congenital heart block and neonatal lupus syndrome: A series of four cases

• Published in: Lupus Vol. 30; no. 2; pp. 307 - 314
• Main Authors: Maltret, Alice, Morel, Nathalie, Levy, Marilyne, Evangelista, Martina, Malekzadeh-Milani, Sophie, Barbet, Patrick, Costedoat-Chalumeau, Nathalie, Bonnet, Damien
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01.02.2021; Sage Publications Ltd
• Subjects: Biopsy; Catheterization; Computed tomography; Congenital diseases; Congenital heart block; Diagnosis; Echocardiography; Hypertension; Hypoxemia; Immunosuppressive agents; Lung diseases; Lupus; Neonates; Pulmonary hypertension; Respiratory diseases; Sildenafil; Systemic lupus erythematosus; Cardiovascular disease; anti-DNA antibodies; pregnancy
• ISSN: 0961-2033; 1477-0962
• DOI: 10.1177/0961203320973073

Objective Neonatal lupus syndrome has multisystemic manifestations among which pulmonary involvement has been rarely reported. We describe the clinical presentation, management, and outcome of a series of four neonates who developed reversible pulmonary hypertension associated with auto-immune congenital complete heart block. Method Data from the French registry of neonatal lupus syndrome were retrospectively reviewed. Results Between 2000 and March 2020, 231 children were included in the French registry, four/73 followed in our institution developed pulmonary hypertension. Diagnosis was suspected on transthoracic echocardiography at a median age of 42 days [range 10-58], and confirmed by right heart catheterization in all; 2 of them where paced at time of diagnosis and 2 were not. All had some degree of hypoxemia and respiratory distress. Hypoxemia was always reversible under O2 et NO. Lung CT demonstrated ground glass anomalies in all. One patient had a lung biopsy consistent with pulmonary hypertension secondary to lung disease. Management included immunosuppressive therapy in 3 associated with sildenafil in 2. Pulmonary hypertension resolved in all at a median age of 4 weeks [range 3-6] after treatment initiation and after one year for the one child who did not receive specific treatment. Conclusion Clinical, hemodynamical, imaging and histological findings advocate for pulmonary hypertension associated with respiratory disease as a rare manifestation of neonatal lupus syndrome.