Hemitruncus Arteriosus Research Articles

An unusual case of left-sided hemitruncus arteriosus associated with aortic arch hypoplasia

An unusual case of left-sided hemitruncus arteriosus associated with aortic arch hypoplasia

Hemitruncus Arteriosus With Anomalous Origin of the Right Coronary Artery From the Right Pulmonary Artery and Unilateral Absence of the Left Pulmonary Artery

Hemitruncus Arteriosus With Anomalous Origin of the Right Coronary Artery From the Right Pulmonary Artery and Unilateral Absence of the Left Pulmonary Artery

Think out of the box: association of left congenital diaphragmatic hernia and abnormal origin of the right pulmonary artery

BackgroundWe report the occurrence of a severe pulmonary hypertension (PH) in a neonate affected by a left congenital diaphragmatic hernia (CDH). PH in this patient was associated with an abnormal origin of the right pulmonary artery from the right brachiocephalic artery. This malformation, sometimes named hemitruncus arteriosus, has to the best of our knowledge never been reported in association with a CDH.Case presentationA male newborn was hospitalized from birth in the neonatal intensive care unit after prenatal diagnosis of a left CDH. Ultrasound examination at 34 weeks of gestational age evaluated the observed-to-expected lung-to-head ratio at 49%. Birth occurred at 38+ 5 weeks of gestational age. Soon after admission, severe hypoxemia, i.e., preductal pulse oximetry oxygen saturation (SpO2) < 80%, prompted therapeutic escalation including the use of high frequency oscillatory ventilation with fraction of inspired oxygen (FiO2) 100% and inhaled nitric oxide (iNO). Echocardiography assessment revealed signs of severe PH and normal right ventricle function. Despite administration of epoprostenolol, milrinone, norepinephrine, and fluid loadings with albumin and 0.9% saline, hypoxemia remained severe, preductal SpO2 inconsistently greater than or equal to 80-85% and post ductal SpO2 lower on average by 15 points. This clinical status remained unchanged during the first 7 days of life. The infant’s clinical instability was incompatible with surgical intervention, while chest X-ray showed a relatively preserved lung volume, especially on the right side. This prompted an additional echocardiography, aimed at searching an explanation of this unusual evolution and found an abnormal origin of the right pulmonary artery, which was confirmed on computed tomography angiography subsequently. A change in the medical strategy was decided, with the suspension of pulmonary vasodilator treatments, the administration of diuretics, and the decrease in norepinephrine dose to decrease the systemic-to-pulmonary shunt. Progressive improvement in the infant respiratory and hemodynamic status enabled to perform CDH surgical repair 2 weeks after birth.ConclusionsThis case recalls the interest of systematic analysis of all potential causes of PH in a neonate with CDH, a condition frequently associated with various congenital malformations.

Hemitruncus arteriosus: a rare congenital cardiac anomaly diagnosed and treated during the neonatal period

No abstract available Sri Lanka Journal of Child Health, 2023: 52(2): 239-241

Hemitruncus derecho en la edad adulta: Un hallazgo poco usual

El hemitruncus arterioso es una cardiopatía congénita rara caracterizada por el origen anómalo de alguna de las ramas de la arteria pulmonar a partir de la aorta ascendente. En la mayoría de los casos su diagnóstico se realiza durante la infancia; además, por su elevada morbimortalidad es extremadamente inusual su evolución asintomática y supervivencia hasta la etapa adulta. Presentamos el caso de un varón de 30 años con antecedente de cierre quirúrgico de persistencia del conducto arterioso en la infancia, que cursó asintomático hasta hace un año, y se le diagnosticó hemitruncus arterioso a partir de episodios recurrentes de hemoptisis.

Total Correction without Cardiopulmonary Bypass of Isolated Left Hemitruncus Arteriosus

Total Correction without Cardiopulmonary Bypass of Isolated Left Hemitruncus Arteriosus

Abnormal Origin of the Right Pulmonary Artery From Ascending Aorta (Hemitruncus Arteriosus).

Hemitruncus arteriosus is a rare congenital deformity that results in early infant mortality. Persistence into adulthood is very unusual and is associated with pulmonary hypertension. We report a case in an adult male with the associated clinical issues.

Hemitruncus Arteriosus: Cardiac Magnetic Resonance Angiography Findings

Hemitruncus arteriosus (HA) is an uncommon congenital cardiac malformation in which one pulmonary artery branch originates from the ascending aorta. A 2-month-old male was referred to our hospital for failure to thrive, cyanosis, and tachypnea. A chest radiograph demonstrated moderate cardiomegaly and peribronchial thickening. Echocardiography demonstrated right atrial and ventricular enlargement and increased gradient (25 mm Hg) in the right pulmonary artery. Cardiac magnetic resonance angiography was performed and clearly revealed typical imaging findings of a right HA which had complex cardiac and vascular anatomy. This case report presents cardiac magnetic resonance angiography findings of HA.

Pulmonary Artery Banding for Hemi-Truncus Arteriosus in Adulthood

Anomalous origin of the right pulmonary artery from the ascending aorta, also called hemi-truncus arteriosus, is a rare congenital cardiovascular malformation. Around 95% of the reported cases with hemi-truncus arteriosus present in infancy. We describe a case of a 41-year-old female presenting with a long-standing history of intermittent haemoptysis. Cardiac catheterisation confirmed the diagnosis of hemi-truncus arteriosus. The definitive treatment of hemi-truncus arteriosus in adulthood is an extremely high-risk procedure. We decided to perform palliative right pulmonary artery banding which represents a simple but effective procedure for the management of this complex condition.

Diagnosis of the Double Aortic Arch and Its Differentiation from the Conotruncal Malformations

PurposeThe clinical and radiological characteristics of the double aortic arch (DAA) and its differentiation from conotruncal malformations (CTM) were reported in order to familiarize pediatric practitioners with these congenital heart diseases.Materials and MethodsFrom July 1994 to December 2006, a total of 6 patients (4 male and 2 female, aged 16 days to 6.5 years) with DAA were enrolled in this retrospective study. The study modalities included chart recordings, plain chest radiographs, barium esophagograms, echocardiograms, cardiac catheterization, cardiac angiograms, surgery, magnetic resonance imaging, and chromosome analysis. Patients with incomplete vascular rings or with right aortic arches and left ligamentum were excluded. In addition, the clinical and radiological profiles of 38 patients with CTM, including dextro-transposition of the great arteries (d-TGA) (n = 28), hemitruncus arteriosus (HTA) (n = 3), type I truncus arteriosus (TA) (n = 4), and the aortopulmonary window (APW) (n = 3), were comparatively reviewed.ResultsAll 6 patients with DAA presented with postprandial choking and respiratory distress that prompted their initial visit to the hospital. One of the 6 patients presented with congestive heart failure, and none with cyanosis. Esophagograms showed indentations in 5 patients with DAA. All patients with d-TGA presented with cyanosis and heart failure, while patients with HTA, type I TA, and APW manifested overt heart failure. Suprasternal and subcostal approaches of the echocardiography may offer diagnositic windows for DAA. As for CTM, parasternal and subcostal approaches could always determine the causality. Cardiac catheterization with angiography comprehensively delineated the pathology.ConclusionIn case of postprandial choking and respiratory distress in neonates and infants, barium esophagograms can indicate the presence of DAA. Diagnosis of DAA and its differentiation from the CTM can be achieved by echocardiography, angiography, or magnetic resonance imaging.

Ascending aortic origin of a branch pulmonary artery--surgical management and long-term outcome.

Ascending aortic origin of a branch pulmonary artery (AOPA, hemitruncus arteriosus) is a rare congenital malformation. While there have been isolated case reports, larger series, relating to long-term outcomes following surgery are few. This article analyses the surgical results of a series of nine patients, over a period of 29 years. Between 1974 and 2003, nine patients [neonates, 6; infants, 3; male, 5; female, 4] were operated on for AOPA. Median age at presentation was 14 days (range birth to 231 days). Six [corrected] patients (group 1) had associated simple lesions like patent ductus arteriosus or right aortic arch. Three patients (group 2) had complex lesions with right ventricular outflow tract obstruction. One patient (group 2) had DiGeorge syndrome. All patients except group 2 presented with congestive cardiac failure and, in addition one had pre-operative coronary ischemia. Diagnosis was established by angiocardiography in two patients and by echocardiography in seven [corrected] The median age at operation was 28 days (range 7-365). Follow-up period ranged from 7 months to 20.5 years (median 9 years). All nine patients had an anomalous right pulmonary artery (RPA) arising from the proximal ascending aorta, while the left branch was of right ventricular origin. All had evidence of pulmonary hypertension or elevated right ventricular pressure pre-operatively. There was no operative mortality. Of eight patients who had direct anastomosis of the RPA to the main pulmonary artery, one required patch enlargement and another required stenting of an anastomotic stenosis. One patient had a RV-RPA conduit, which required replacement 8, 13, and 14 years later. At follow-up, all patients were alive. All patients in group 1 had normal haemodynamic function and were in NYHA class I. In group 2, all were in NYHA class II with evidence of right ventricular hypertrophy. Four patients had post-operative ventilation-perfusion scans which showed satisfactory perfusion to both lungs. Early surgery is indicated in this lesion and is compatible with good long-term outlook. Surgical repair should not be deferred for corrective procedures of associated cardiac anomalies.

Left hemitruncus in adulthood: diagnostic role of magnetic resonance imaging

The diagnosis of left hemitruncus and large patent ductus arteriosus was made by magnetic resonance imaging in an adult patient with recurrent haemoptysis and dyspnoea on exertion. Previous cardiac catheterization and echocardiography failed to establish the complete diagnosis. Magnetic resonance imaging using spin-echo and gradient-echo pulse sequences is a useful imaging modality to evaluate anatomical and functional abnormalities in patients with complex congenital heart disease.

Truncus Arteriosus

Truncus arteriosus and hemitruncus arteriosus are congenital cardiac anomalies that present with congestive heart failure during the early months of life. As a consequence of their pathophysiology, patients with these anomalies may be prone to develop myocardial ischemia during general anesthesia. The following cases demonstrate intraoperative electrocardiographic detection of myocardial ischemia prior to surgical repair, as well as successfull intraoperative (prebypass) management