Potts Shunt in Children with Familial Primary Pulmonary Hypertension: a Case Report and Brief Literature Review

The major concern about familial pulmonary arterial hypertension (FPAH) is the development of the right heart failure which ultimately leads to a sudden death. Despite recent advances in pharmacological therapy, the mortality rate in children is still high, emphasizing the importance of novel treatments. Moreover, there is yet no definitive treatment for children with severe pulmonary hypertension. The consequences of right heart failure led to the proposal of a surgical approach, the Potts shunt, to decompress the right ventricle, providing shunting of deoxygenated blood to lower extremities and improving overall cardiac output. This surgical technique creates an anastomosis between the left pulmonary artery and the descending aorta, providing a palliative treatment to off-load the right ventricle.

We report the case of an 11-year-old girl with familial pulmonary arterial hypertension and right ventricular failure who benefited of Potts shunt with good mid-term results. The patient was diagnosed at the age of 5 when she had history of fainting and persistent syncope. The patient was under medical treatment therapy with bosentan, sildenafil, acetylsalicylic acid (ASA), warfarin for six years. The results of molecular genetic testing which was confirmed with direct sequencing of bone morphogenetic protein receptor type 2 (BMPR2) gene revealed a heterozygous pathogenic mutation. Since she was diagnosed with PAH, she lost her grandmother, an aunt, and her father to pulmonary arterial hypertension (PAH). Her 14-year-old sister also had mutated BMPR2 gene without developing FPAH.

The Potts shunt provides an interventional step for palliation of patients with familial pulmonary hypertension and severe right heart failure refractory to medical treatment which opened the door to the possibility of lung transplantation in the future. We didn’t see any complications within 6 years after placing Potts shunt.