Congenital Heart Disease with Severe PH Can Be Treated Surgically

In a new retrospective study, researchers show that a procedure called “palliative Senning” improves arterial oxygenation and life quality in children with congenital heart diseases and severe pulmonary hypertension (PH). The research paper, entitled “Palliative Senning in the Treatment of Congenital Heart Disease with Severe Pulmonary Hypertension,” was published in Arquivos Brasileiros de Cardiologia by researchers at the Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da USP, in São Paulo, Brazil.

The transposition of the great arteries (TGA) is the most common of cyanotic cardiopathies, where the pulmonary artery and the aorta are switched in position, leading to poorly oxygenated blood being pumped from the heart to the rest of the body. According to the Centers for Disease Control and Prevention (CDC), TGA is diagnosed in 5 out of every 10,000 babies born in U.S. Children with TGA and with ventricular septal defect (VSD) were first treated with palliative surgery decades ago, and such surgery has since been used in other congenial heart diseases to contain pulmonary hypertension (PH) and improve arterial oxygen saturation. Although most babies diagnosed with TGA with VSD or Taussig-Bing double-outlet right ventricle (DORV) do not progress to pulmonary vascular disease, those who do not undergo surgery early in life, or even before birth, usually develop PH. This progression is still a major concern in the treatment of TGA patients.

In this study, researchers evaluated the results of palliative surgical procedures done from 1991 to 2014 on 21 patients, ranging from 1 month to nearly 11 years old, with a diagnosis of TGA with VSD (81%) and Taussig-Bing double-outlet right ventricle (19%), experiencing severe PH. Medical record evaluation shows that the initial mortality rate was 47% (10 patients). Postoperative comorbidities that did not result in death included pulmonary hypertensive crisis, pneumonia, acute renal failure (ARF), pulmonary congestion, total atrioventricular block (TAVB), and junctional rhythm. There was a mean increase in arterial oxygen saturation from 62.1% to 92.5%, and a mean reduction in hematocrit from 49.4% to 36.3%, in assessments made one year after hospital discharge.

After 19 years of follow-up, the survival rate was better than in earlier studies, accounting for 52.3%. Researchers also noted that seven of the 10 deaths occurred more than 20 years ago, when medical resources were less developed. Functional assessment according to the New York Heart Association (NYHA) revealed that all patients improved functional class, with preservation of ventricular functioning.

Researchers conclude that the palliative Senning procedure showed numerous benefits for patients with TGA and VSD or Taussig-Bing DORV, who had severe PH and were considered inoperable, increasing their survival rate. They also found that pulmonary lesions were more frequently in patients who underwent surgery after the age of 12 months, suggesting the procedure is most effective when done early in life.