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Truncus arteriosus

Rouven Kubicki, Brigitte Stiller, Jochen Grohmann
Pseudoaneurysm formation is a rare but potentially life-threatening complication after surgical repair of congenital heart disease. We present a boy with truncus arteriosus communis 14 years after homograft placement in pulmonary position. On follow-up, he presented progressive chronic homograft degeneration. Moreover, a large pseudoaneurysm in the right ventricular outflow tract was surprisingly depicted. We opted for a two-stage interventional approach.
2016: SpringerPlus
Richard Van Praagh
The situs, or pattern of anatomic organization, of the subarterial infundibulum and of the great arteries and the degree of development of the subarterial infundibulum largely determine whether the great arteries are normally or abnormally related. There are 2 types of situs: solitus (normal) and inversus (a mirror image of solitus). Situs ambiguus means that the pattern of anatomic organization is uncertain or unknown. Infundibular development varies from absent, to atretic, to severely stenotic, to mildly or moderately stenotic; great arteries are solitus normally related or inversus normally related, respectively...
August 12, 2016: American Journal of Cardiology
Nabil Hussein, Simone Speggiorin, Frances Bu'Lock, Antonio F Corno
An intramural coronary artery in the setting of truncus arteriosus (common arterial trunk) is an uncommon association. Following an uneventful surgical repair, a neonate developed a low cardiac output state deteriorating into cardiac arrest shortly after arrival into the intensive care unit, requiring extracorporeal membrane oxygenation support. Echocardiography and angiography showed occlusion of the left coronary artery, prompting emergency surgical reexploration. A "slit-like" orifice with an intramural left coronary artery was successfully unroofed, allowing full recovery...
September 12, 2016: World Journal for Pediatric & Congenital Heart Surgery
Akihisa Furuta, Mitsugi Nagashima, Takeshi Hiramatsu, Goki Matsumura, Kenji Yamazaki
The unilateral absence of a proximal pulmonary artery (UAPPA) is rare and is most frequently accompanied by cardiovascular anomalies such as tetralogy of Fallot or septal defects. We report a patient with truncus arteriosus with UAPPA in which we performed a two-stage surgical repair. During the first palliative operation, a right modified Blalock-Taussig shunt was constructed to develop the hypoplastic right pulmonary artery. At 10 months, the patient underwent patch closure of a ventricular septal defect with integration of both pulmonary arteries, and reconstruction of the right ventricular outflow tract using a conduit...
September 9, 2016: Journal of Cardiac Surgery
Pei Ma, Shi Gu, Ganga H Karunamuni, Michael W Jenkins, Michiko Watanabe, Andrew M Rollins
Cardiac neural crest cell (CNCC) ablation creates congenital heart defects (CHDs) that resemble those observed in many syndromes with craniofacial and cardiac consequences. The loss of CNCCs causes a variety of great vessel defects, including persistent truncus arteriosus and double outlet right ventricle. However, due to the lack of quantitative volumetric measurements, less severe defects, such as great vessel size changes and valve defects have not been assessed. Also poorly understood is the role of abnormal cardiac function in the progression of CNCC-related CHDs...
August 19, 2016: American Journal of Physiology. Heart and Circulatory Physiology
Billie-Jean Martin, Tara B Karamlou, Sarah Tabbutt
OBJECTIVES: The objectives of this review are to describe the anatomy, pathophysiology, perioperative therapeutic strategies, and operative procedures for patients with anomalous pulmonary venous connections and truncus arteriosus. DATA SOURCE: MEDLINE and PubMed. CONCLUSIONS: An understanding of the anatomy and pathophysiology of anomalous pulmonary venous connections and truncus arteriosus is essential for the optimal perioperative management of these complex and challenging congenital lesions...
August 2016: Pediatric Critical Care Medicine
Uma Pandey, Neeraj Kumar Agrawal, Shilpa Agrawal, Shuchita Batra
AIM: The study was done to determine the maternal and fetal outcome of pregnancies complicated by maternal diabetes either Gestational Diabetes Mellitus (GDM) or preexisting (type 1 or type 2) diabetes over a period from March 2011 to Feb 2013 in a tertiary care hospital, Varanasi. METHODS: This is a retrospective audit of the maternal and fetal outcome of women who presented to the Sir Sundar Lal Hospital, Department of Obstetrics and Gynaecology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India from March 2011 to Feb 2013, with GDM or pre-existing type 1 or type 2 Diabetes with pregnancy...
August 2016: Journal of Obstetrics and Gynaecology of India
W Aaron Kay
Thoracic aortic dilation (AD) has commonly been described in conotruncal defects (CTDs), such as tetralogy of Fallot, double outlet right ventricle and transposition of the great arteries, and truncus arteriosus. Several theories for this have been devised, but fairly recent data indicate that there is likely an underlying histologic abnormality, similar to that seen in Marfan and other connective tissue disease. The majority of aortic dissection in the general population occurs after the age of 45 years, and there have been very few case reports of aortic dissection in CTD...
2016: Frontiers in Cardiovascular Medicine
Angira Patel, John M Costello, Carl L Backer, Sara K Pasquali, Kevin D Hill, Amelia S Wallace, Jeffrey P Jacobs, Marshall L Jacobs
BACKGROUND: Among patients with congenital heart disease (CHD), the coexistence of noncardiac congenital anatomic abnormalities (NC), genetic abnormalities (GA), and syndromes (S) may influence therapeutic strategies and outcomes. The appreciated prevalence of these abnormalities has risen because increased screening and improved diagnostic precision enable identification of these comorbidities in a larger fraction of neonates with CHD. We examined the contemporary prevalence and distribution of NC/GA/S across diagnostic groups among neonates undergoing cardiac operations using a large nationally representative clinical registry...
June 17, 2016: Annals of Thoracic Surgery
Daniel Hurtado-Sierra, Catalina Sánchez-Cornelio, Clara A Vázquez-Antona, Alfonso Buendía-Hernández
No abstract text is available yet for this article.
July 2016: Archivos de Cardiología de México
Arun Sharma, Sarv Priya, Priya Jagia
Persistent truncus arteriosus is a rare congenital cardiac disease with variable presentation. The exact preoperative diagnosis and delineation of anatomy are very important because the optimal timing and procedure for truncus arteriosus repair are decided on the basis of the morphological characteristics. Moreover, the presence of associated anomalies influences the surgical outcome and mortality in these patients. Dual-source computed tomographic evaluation with three-dimensional post-processing is highly valuable for delineating its precise morphology and to identify and characterize the associated anomalies...
July 2016: Japanese Journal of Radiology
Sachin Talwar, Palleti Rajashekar, Saurabh Kumar Gupta, Gurpreet Singh Gulati, Balram Airan
We report a 14-month-old child with persistent truncus arteriosus and crossed pulmonary arteries. The potential advantage of crossed pulmonary artery arrangement in achieving surgical correction is discussed.
June 2016: Annals of Thoracic Surgery
Zhandong Zeng, Hongwei Zhang, Fengli Liu, Ning Zhang
Congenital heart defects (CHD) affect approximately 7% of infants, and account for 3% of all infant deaths. CHD is most often caused by the defects associated with ductus arteriosus, which is a vessel that usually closes shortly after birth. The types of CHD include tetralogy of fallot, hypoplastic left heart syndrome, pulmonary atresia, total anomalous pulmonary venous return, transposition of great arteries, tricuspid atresia and truncus arteriosus. There are some risk factors that can increase the chance of a fetus developing CHD such as prematurity, an existing CHD in a first-degree relative, genetic syndromes, infections in utero, maternal drug consumptions and disorders...
May 2016: Experimental and Therapeutic Medicine
Tai Fuchigami, Masahiko Nishioka, Toru Akashige, Kazuhiro Takahashi, Taisuke Nabeshima, Nobuhiro Nagata
A neonate, born after 29 weeks and 2 days gestation (weight 1015 g), was diagnosed with truncus arteriosus and interrupted aortic arch. On postnatal day 30 (1378 g), we performed bilateral pulmonary artery banding. On postnatal day 107 (3024 g), we carried out aortic arch repair and a Rastelli-type procedure. During follow-up, branch pulmonary artery and ascending aortic stenosis was observed. At 15 months, surgical angioplasty of the ascending aorta and bilateral branch pulmonary arteries was undertaken using the Lecompte maneuver...
May 5, 2016: Asian Cardiovascular & Thoracic Annals
Seiji Asagai, Kei Inai, Tokuko Shinohara, Hirofumi Tomimatsu, Tetsuko Ishii, Hisashi Sugiyama, In-Sam Park, Mitsugi Nagashima, Toshio Nakanishi
OBJECTIVES: This study aimed to analyze long-term survival and functional outcomes after truncus arteriosus repair in a single institution with more than 40 years of follow-up. METHODS: Medical records were analyzed retrospectively in 52 patients who underwent the Rastelli procedure for truncus arteriosus repair between 1974 and 2002. Thirty-five patients survived the initial repair. The median age at the initial operation was 2.8 months (range, 0.1-123 months) and the body weight was 3...
April 29, 2016: Congenital Heart Disease
Tamara McMillan, Rose Girgis, Elizabeth A C Sellers
BACKGROUND: Neonatal diabetes is a rare form of monogenic diabetes with onset in the first six months of life occurring in 1/100,000 to 1/400,000 births. Both permanent and transient forms have been described. Permanent neonatal diabetes results predominantly from mutations in the KCNJ11 and ABCC8 genes. Less frequently, mutations of the GATA6 gene, located on chromosome 18 cause a form of permanent neonatal diabetes resulting from pancreatic hypoplasia or agenesis. Other anomalies associated with mutations of this gene have also been reported, most commonly congenital heart disease...
2016: BMC Medical Genetics
Yanqing Wu, E Albert Reece, Jianxiang Zhong, Daoyin Dong, Wei-Bin Shen, Christopher R Harman, Peixin Yang
BACKGROUND: Maternal type 1 and 2 diabetes mellitus are strongly associated with high rates of severe structural birth defects, including congenital heart defects. Studies in type 1 diabetic embryopathy animal models have demonstrated that cellular stress-induced apoptosis mediates the teratogenicity of maternal diabetes leading to congenital heart defect formation. However, the mechanisms underlying maternal type 2 diabetes mellitus-induced congenital heart defects remain largely unknown...
September 2016: American Journal of Obstetrics and Gynecology
Takahisa Sakurai, Hajime Sakurai, Koji Yamana, Toshimichi Nonaka, Rei Noda, Ryohei Otsuka, Takuya Osawa
OBJECTIVES: Bilateral pulmonary artery banding (bil-PAB) has been developed as a part of a hybrid procedure for hypoplastic left heart syndrome (HLHS). We use this procedure for palliation of complex congenital heart disease, particularly in patients with arch anomaly. We reviewed our experience with bil-PAB. METHODS: Sixty-six consecutive cases between 2003 and 2014 were reviewed retrospectively. RESULTS: Fifty-one patients (77%) had single ventricle physiology (HLHS: 37, HLHS variant: 13, others: 1)...
March 31, 2016: European Journal of Cardio-thoracic Surgery
Wen Ruan, Yee Jim Loh, Kenneth Wei Qiang Guo, Ju Le Tan
BACKGROUND: Persistent truncus arteriosus is a rare congenital condition with which survival into adulthood is dismal without surgery. This is the oldest patient reported to our knowledge demonstrating the feasibility of assessing operability in persistent truncus arteriosus with unilateral pulmonary stenosis, and performing full corrective surgery in adulthood. CASE PRESENTATION: We report a Chinese male with successful correction of Type I persistent truncus arteriosus at 33 years of age...
2016: Journal of Cardiothoracic Surgery
Madan Mohan Maddali, John Valliattu, Pranav Subbaraya Kandachar, Eapen Thomas, Arora Ram Nishant
During the surgical repair of a truncus arteriosus with aortic arch interruption and pulmonary artery origin stenosis, a Contegra conduit was implanted as part of reconstruction of the pulmonary artery in a small infant. There was a mismatch between the conduit size and the patient that resulted in protrusion of the conduit between the sternal edges. To accommodate the conduit inside the thoracic cavity, traction was applied to the sternum that was gradually released over a period of time guided by transesophageal echocardiography-derived cardiac output data, as well as continuous hemodynamic parameters...
May 2016: Journal of Cardiac Surgery
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