Carlos M Mery1, Kimberly M Molina2, Rajesh Krishnamurthy3, Charles D Fraser4, Henri Justino5. 1. Division of Congenital Heart Surgery, Texas Children's Hospital; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Tex. Electronic address: cmmery@texaschildrens.org. 2. Section of Pediatric Cardiology, Primary Children's Medical Center, University of Utah, Salt Lake City, Utah. 3. Division of Pediatric Radiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Tex. 4. Division of Congenital Heart Surgery, Texas Children's Hospital; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Tex. 5. Lillie Frank Abercrombie Section of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Tex.
Abstract
OBJECTIVE: Ductal origin of a pulmonary artery (DOPA) is commonly misdiagnosed as agenesis of a pulmonary artery (PA), which may result in inadequate treatment. The objective is to describe the results of resuscitation of unilateral DOPA. METHODS: This study is a retrospective review of all patients with unilateral DOPA who underwent PA resuscitation at Texas Children's Hospital from 1993 to 2012. Patients with other cardiac or contralateral lung anomalies were excluded. RESULTS: Ten patients, median age 2 years (range, 3 days to 9 years), with unilateral DOPA were included. Symptoms were present in 6 patients. Cardiac catheterization was performed in all and showed a patent duct or a ductal stump in most patients and a small PA on wedge angiography of the pulmonary veins. Two patients underwent single-stage centralization. The other 8 underwent ductal stenting (n=2) or a systemic-to-PA shunt (n=6) as the first stage before centralization. The 2 patients with ductal stenting developed pulmonary edema. The 2 patients with a cryopreserved vein shunt developed early thrombosis requiring reintervention. Nine patients have undergone centralization. Six patients have required further interventional procedures. There have been no deaths. Symptoms and lung hypoplasia have improved in all patients. Median relative lung perfusion at follow-up was 26% (range, 12%-46%) with significant improvement in the size of the affected PA. CONCLUSIONS: PA resuscitation is effective at restoring flow to the affected lung resulting in improved diameter of the PA, lung growth, and resolution of symptoms. PA resuscitation should be considered in all children with DOPA, including those beyond infancy.
OBJECTIVE: Ductal origin of a pulmonary artery (DOPA) is commonly misdiagnosed as agenesis of a pulmonary artery (PA), which may result in inadequate treatment. The objective is to describe the results of resuscitation of unilateral DOPA. METHODS: This study is a retrospective review of all patients with unilateral DOPA who underwent PA resuscitation at Texas Children's Hospital from 1993 to 2012. Patients with other cardiac or contralateral lung anomalies were excluded. RESULTS: Ten patients, median age 2 years (range, 3 days to 9 years), with unilateral DOPA were included. Symptoms were present in 6 patients. Cardiac catheterization was performed in all and showed a patent duct or a ductal stump in most patients and a small PA on wedge angiography of the pulmonary veins. Two patients underwent single-stage centralization. The other 8 underwent ductal stenting (n=2) or a systemic-to-PA shunt (n=6) as the first stage before centralization. The 2 patients with ductal stenting developed pulmonary edema. The 2 patients with a cryopreserved vein shunt developed early thrombosis requiring reintervention. Nine patients have undergone centralization. Six patients have required further interventional procedures. There have been no deaths. Symptoms and lung hypoplasia have improved in all patients. Median relative lung perfusion at follow-up was 26% (range, 12%-46%) with significant improvement in the size of the affected PA. CONCLUSIONS: PA resuscitation is effective at restoring flow to the affected lung resulting in improved diameter of the PA, lung growth, and resolution of symptoms. PA resuscitation should be considered in all children with DOPA, including those beyond infancy.
Authors: Konstantinos Dimopoulos; Gerhard-Paul Diller; Alexander R Opotowsky; Michele D'Alto; Hong Gu; George Giannakoulas; Werner Budts; Craig S Broberg; Gruschen Veldtman; Lorna Swan; Maurice Beghetti; Michael A Gatzoulis Journal: J Am Heart Assoc Date: 2018-07-04 Impact factor: 5.501