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Embryologically, incomplete conotruncal septation with resultant single aortopulmonary trunk and defective ventricular septation defines the congenital cardiac lesion known as persistent truncus arteriosus (PTA). Torrential pulmonary blood flow is inevitable when this rare lesion is further compounded by patency of the arterial ductus. Such was the case of a patient who presented with fast breathing, reduced suck, darkening of the tongue, and extremities. Urgent echocardiographic diagnosis was PTA (Type A1) with patent ductus arteriosus and pulmonary hypertension and left ventricular systolic dysfunction. [ABSTRACT FROM AUTHOR]

The article describes the concurrent occurrence of persistent truncus arteriosus with intact interventricular septum, left ventricular hypoplasia, patent foramen ovale, atrioventricular fistula, and coronary artery malformations in a male Holstein calf. Topics include severe to moderate subendocardial fibrosis in the left ventricle, genetic factors and familial occurrence in keeshond dogs, and inhibition of the migration of cardiac crest cells.

Abstract: Anomalies of conotruncal septation are rare in dogs and uncommon in humans. Congenital conotruncal defects most commonly reported in veterinary medicine include aorto-pulmonary window and persistent truncus arteriosus. We report a case of an anomalous vessel connecting the ascending aorta to the right pulmonary artery causing left-to-right shunting, left-sided volume overload, and pulmonary overcirculation. Transesophageal echocardiography, cardiac catheterization, and contrast-enhanced computed tomography assisted in the diagnosis and facilitated the surgical correction of the anomalous vessel. The authors hypothesize this defect represents an unusual anomalous vessel connecting the ascending aorta to the right pulmonary artery. [Copyright &y& Elsevier]

Objective: Postoperative atrial fibrillation is the most common complication after cardiac surgery. A variety of postoperative atrial fibrillation risk factors have been reported, but study results have been inconsistent or contradictory, particularly in patients with preexisting atrial fibrillation. The incidence of postoperative atrial fibrillation was evaluated in a group of 10,390 patients undergoing cardiac surgery among a comprehensive range of risk factors to identify reliable predictors of postoperative atrial fibrillation. Methods: This 20-year retrospective study examined the relationship between postoperative atrial fibrillation and demographic factors, preoperative health conditions and medications, operative procedures, and postoperative complications. Multivariate logistic regression models were used to evaluate potential predictors of postoperative atrial fibrillation. Results: Increasing age, mitral valve surgery (odds ratio = 1.91), left ventricular aneurysm repair (odds ratio = 1.57), aortic valve surgery (odds ratio = 1.52), race (Caucasian) (odds ratio = 1.51), use of cardioplegia (odds ratio = 1.36), use of an intraaortic balloon pump (odds ratio = 1.28), previous congestive heart failure (odds ratio = 1.28), and hypertension (odds ratio = 1.15) were significantly associated with postoperative atrial fibrillation. The non-linear relationship between age and postoperative atrial fibrillation revealed the acceleration of postoperative atrial fibrillation risk in patients aged 55 years or more. In patients undergoing coronary artery bypass grafting, increasing age and previous congestive heart failure were the only factors associated with a higher risk of postoperative atrial fibrillation. There was no trend in incidence of postoperative atrial fibrillation over time. No protective factors against postoperative atrial fibrillation were detected, including commonly prescribed categories of medications. Conclusions: The persistence of the problem of postoperative atrial fibrillation and the modest predictability using common risk factors suggest that limited progress has been made in understanding its cause and treatment. [Copyright &y& Elsevier]

This article present a case report involving a three-week-old lamb with persistent truncus arteriosus (PTA), a serious cardiac malformation. PTA was characterized by the presence of a common arterial trunk originating from the right ventricle and overriding a ventricular septal defect. A rupture of the PTA with subsequent cardiac tamponade was the cause of the lamb's death. Histologic examination identified a dissecting aneurysm with elastic fiber fragmentation in the wall of the PTA.

Abstract: A 5-month-old male domestic cat presented with a history of rapid, heavy breathing and cyanosis after exercise. Physical examination showed an abnormal respiratory pattern with an increased rate and stress-induced cyanosis. Auscultation revealed tachycardia and a grade 5/6 systolic murmur best heard over the left base. Radiographs showed evidence of right atrial and ventricular enlargement with distended pulmonary vessels and an enlarged ascending aorta. An echocardiographic examination revealed a dilated right atrium, eccentric right ventricular hypertrophy and an overriding aorta associated with a large ventricular septal defect (VSD). The pulmonary trunk could not be identified by echocardiography. Doppler and saline contrast studies showed large right-to-left shunting through the VSD. These findings were compatible with persistent truncus arteriosus, which was confirmed at necropsy. [Copyright &y& Elsevier]

Abstract: Crohn’s disease (CD) is a chronic granulomatous inflammatory bowel disease which may also involve the extraintestinal organs such as joints, liver, skin and perianal tissue. Involvement of the nasal cavity is quite rare in CD. This case report presents a 28-year-old girl with CD and saddle nose deformity, alar collapse and palatal perforation as extraintestinal manifestations of the disease in addition to persistent truncus arteriosus-type 4. [Copyright &y& Elsevier]

We report a case of truncus arteriosus type II with a large outlet ventricular septal defect and a hypoplastic left ventricle. The first-stage palliation was the removal of both branch pulmonary arteries from the ascending aorta, patch augmentation of the pulmonary artery confluence, and modified Blalock-Taussig shunt at age 1 month. The second stage palliation was a bidirectional Glenn at 6 months. The final stage was an extracardiac Fontan at age 3 years. Eight years later, the patient is doing well, with an unobstructed Fontan pathway and mild-to-moderate truncal valve insufficiency. [Copyright &y& Elsevier]

The article presents a study of congenital cardiac anomaly known as tetralogy of Fallot (TOF), which is characterized by right ventricular outflow tract obstruction caused by subpulmonary stenosis, a ventricular septal defect, and right ventricular hypertrophy. It claims that diagnosis of TOF with pulmonary atresia relies on the presence of a pulmonary valve remnant by clinical imaging. However, if this develops embryonically this would not determine persistent truncus arteriosus from TOF.

In humans, TBX1 is the major candidate gene for DiGeorge (del22q11.2) syndrome, which includes heart malformations such as tetralogy of Fallot and Persistent Truncus Arteriosus (PTA). In Tbx1 null embryos, Second Heart Field (SHF) cardiac progenitor cell numbers are decreased leading to a hypoplastic outflow tract and PTA. Recently, we have shown that the Tbx1 phenotype is associated with reduction of a specific progenitor cell population that normally contributes to myocardium at the base of the pulmonary trunk. The Tbx1 mutant ventricular outlet thus has a predominantly subaortic identity supported by the presence of a single outflow valve with three leaflets. In addition, we demonstrated that coronary artery patterning is abnormal in Tbx1-nulls. Proximal coronary arteries course abnormally across the ventral region of mutant hearts and left and right arteries branch to the right/ventral sinus of the common outlet. Coronary artery patterning defects are observed at early developmental stages at the level of the coronary plexus suggesting that SHF derived cells influence the cellular and molecular events responsible for the distribution and branching of proximal coronary arteries. We have identified Semaphorin3c as a Tbx1-dependent gene expressed in subpulmonary myocardium. However, Sema3c-null embryos do not show major coronary artery defects suggesting that Sema3c function overlaps with that of other genes affected in Tbx1 mutant embryos. We are now investigating the distribution and patterning of additional vascular guidance molecules as well as the distribution of neural crest cells and cardiac cushions which play critical roles in outflow tract development in wild type mice. Our results provide new insights into the association between conotruncal defects and coronary artery anomalies and implicate SHF derived cells in coronary artery patterning. Ongoing research in collaboration with Necker Hospital (Paris) aims to investigate whether specific coronary artery anomalies are associated with PTA in DiGeorge syndrome. [Copyright &y& Elsevier]