Pulmonary artery damage is difficult to estimate in a patient with one pulmonary artery from the aorta, and the pulmonary artery of anomalous origin is usually damaged. We describe a newborn patient with anomalous origin of the right pulmonary artery from the aorta who presented with significant lung perfusion at the anastomotic site 6 months postoperatively; the left/right perfusion ratio was 10:90 on a scintigram. The unbalanced left/right lung perfusion gradually improved over a number of years. In a newborn patient with anomalous origin of one pulmonary artery from the aorta, unbalanced lung perfusion may improve.
Introduction: Thebesian veins (ThVs) are coronary veins that drain directly into the atrial or ventricular chamber. Angiographically detectable ThVs are a rare finding sometimes associated with myocardial ischemia due to coronary steal and volume overload of ventricles in adults. However, there are limited data concerning ThVs in the setting of congenital heart disease. The purpose of this study is to demonstrate presence, character, and changes with growth of ThVs in adult patients late after Fontan operation. Methods: This study is single-center retrospective review of adults with Fontan circulation patients who had undergone cardiac catheterization for routine Fontan surveillance between 2009-2019. We assessed the presence of angiographically detectable ThVs from ascending aortography. After identification of the characteristic cases, we examined the ventricular morphology, the portion of ThVs drainage, and compared with previous findings of angiograms. We also reviewed clinical manifestation and findings of exercise stress ECG of the patients. Pulmonary atresia with intact ventricular septum was excluded because abnormal coronary arterial communications have been well documented in this condition. Results: Among 75 Fontan patients who had undergone catheterization, significant ThVs were observed in a total of 12 cases (16%). There were 6 right-dominant single ventricle, 4 left-dominant single ventricle, and 2 unbalanced two ventricles. In all cases, including left-dominant single ventricle, all ThVs drained into right ventricle cavity, never into left ventricle. The patients who underwent serial catheterizations demonstrated that ThVs dilation and tortuosity progressed over time. Among 6 patients who had underwent exercise stress ECG, only 1 patient showed ST segment depression, however, none had clinical history of chest pain. Conclusions: ThVs are common and progress in adult patients late after Fontan operation. Although the clinical significance of these findings remains uncertain, particular attention should be given to myocardial ischemia and volume overload for the future of adult Fontan patients who have prominent ThVs.
Pulmonary arteriovenous malformations (PAVM) are mostly associated with hereditary hemorrhagic telangiectasia (HHT). We report two pediatric patients diagnosed with HHT on the basis of PAVM who died because of cerebral and spinal AVM rupture. Two 4-year-old boys were diagnosed with PAVM due to hypoxia. Case 1: This patient underwent transcatheter embolization for PAVM four times, but the efficacy was limited. He suffered a complication in the form of cerebral AVM and was treated conservatively. We diagnosed him with HHT because of recurrent epistaxis, telangiectasia of the skin, and AVM. Genetic tests revealed a mutation in the gene for endoglin. At the age of 7 years and 5 months, he died of cerebral AVM rupture. Case 2: Transcatheter embolization could not be performed because of multiple, diffuse PAVM. The patient was complicated by spinal AVM and treated conservatively. We suspected HHT because of telangiectasia of the skin and AVM. At the age of 5 years and 6 months, he died of spinal AVM rupture. HHT is vascular dysplasia that is expressed in multiple organs, and sepsis is the main cause of death. We tend to treat PAVM. But because cerebral and spinal AVM are common as the cause of death, we should check for other forms too, such as cerebral and gastrointestinal AVM, and continue long-term follow-up with concomitant consultation. However, untreatable or unexpected manifestations may occur in these cases, making HHT difficult to treat.
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