One Truncal Valve or Two Semilunar Valves: An Unusual Case of Truncus Arteriosus.
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ABSTRACT:
SUBMITTER: Loke YH
PROVIDER: S-EPMC6058763 | biostudies-literature | 2018 Feb
REPOSITORIES: biostudies-literature
Publications
One Truncal Valve or Two Semilunar Valves: An Unusual Case of Truncus Arteriosus.
Loke Yue-Hin YH Sinha Lok L Olivieri Laura J LJ Jonas Richard A RA Cross Russell R RR Schidlow David N DN
CASE (Philadelphia, Pa.) 20171115 1
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Project description:Stenosis of systemic semilunar valve in cyanotic congenital heart defects is rare. It can happen in truncus arteriosus with truncal valve stenosis and the very rare anomaly of tetralogy of fallot with aortic valve stenosis. Here we describe a neonate with pulmonary atresia, ventricular septal defect and associated aortic valve stenosis and discuss the points of differentiation from truncus arteriosus.
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Project description:Persistent truncus arteriosus is a rare congenital cardiac malformation. It is characterized by a single great artery arising from the heart which supplies the aorta, the origin of coronary arteries and pulmonary arteries. Without surgery, prognosis is poor and 90% of these patients die before one year of age. We report a rare case of an asymptomatic 35-year-old woman with uncorrected persistent truncus arteriosus and hypoplastic right and left pulmonary arteries. Hypoplastic branch pulmonary arteries prevented the development of severe pulmonary arterial hypertension.
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Project description:PHACE syndrome is a rare neurocutaneous syndrome that describes the association of large segmental infantile haemangioma involving the head and neck, along with other systemic anomalies. Complex congenital heart disease has rarely been reported in this syndrome. We present a report of a patient with PHACE syndrome and truncus arteriosus.
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Project description:BackgroundTruncus arteriosus (TA) or persistent arterial trunk describes the presence of a solitary arterial trunk arising from the base of the heart, supported by a common ventriculoarterial junction. The trunk gives rise to the coronary arteries, systemic arteries, and at least one pulmonary artery. Truncus arteriosus is a rare congenital cardiac disease, and even rarer is the absence of ventricular septal defect.Case summaryWe report a case of a 2-day-old infant who presented with cyanosis and a cardiac murmur. He was diagnosed with TA with intact ventricular septum (IVS), as well as crossed pulmonary arteries which was identified on pre-operative imaging. We describe the surgical management and short-term follow-up.DiscussionOur clinical case demonstrates a unique diagnosis and management of TA with IVS identified by pre-operative imaging with good surgical outcome.
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Project description:Truncus arteriosus (TrA) type A3, according to the Van Praagh (VP) classification, cannot be classified under the Collett and Edwards (C-E) system. In this rare anomaly, postnatal closure of the ductus arteriosus can cause unilateral pulmonary artery obstruction; hence, prenatal diagnosis and early confirmatory postnatal diagnosis are important. This case was referred to our hospital for suspected fetal heart disease at 29 weeks' gestation. TrA C-E type 1 was diagnosed by fetal echocardiography, with a right-sided aortic arch, absent inferior vena cava, and azygos continuation. The neonate was delivered vaginally at 41 weeks' gestation. Postnatal echocardiography showed a right-sided aortic arch with the right pulmonary artery originating from the common arterial trunk and the left pulmonary artery originating from the brachiocephalic artery. The diagnosis was TrA VP type A3, with a right-sided aortic arch and left-sided ductus arteriosus. Patency of the left-sided ductus arteriosus was maintained with prostaglandin E1.alpha-cyclodextrin. Right pulmonary artery banding was performed 3 days after birth. The Rastelli procedure was performed when the patient was 2 months old and weighed 4.2 kg. Delayed diagnosis of VP type A3 can cause unilateral pulmonary artery disconnection; hence, timely and accurate diagnosis is warranted to ensure stable disease management. <Learning objective: Delayed diagnosis of truncus arteriosus (TrA) Van Praagh (VP) type A3 can cause unilateral pulmonary artery disconnection; thus, we should consider both Collett and Edwards and VP classifications, including a ductus arteriosus contralateral to the aortic arch, during TrA diagnosis. Furthermore, to prevent the risks associated with an open-heart surgery in the neonatal period in such cases, pulmonary blood flow can be controlled by maintaining the ductus arteriosus with prostaglandin E1.alpha-cyclodextrin and unilateral pulmonary artery banding.>.
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Project description:Truncus arteriosus (TA) is a rare congenital heart disease defined as a single arterial vessel arising from the heart that gives origin to the systemic, pulmonary and coronary circulations. The truncal valve in majority of the cases is tricuspid though quadricuspid and bicuspid valves have been reported. Patients with TA typically have a large nonrestrictive sub truncal ventricular septal defect. Survival of these infants beyond 1-year is uncommon. Here, we report a unique case of 12-year-old female patient with persistent TA who underwent surgical repair by using transesophageal echocardiography as a monitoring device during the perioperative management.
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Project description:ObjectivesNeither heart valve repair methods nor current prostheses can accommodate patient growth. Normal aortic and pulmonary valves have 3 leaflets, and the goal of valve repair and replacement is typically to restore normal 3-leaflet morphology. However, mammalian venous valves have bileaflet morphology and open and close effectively over a wide range of vessel sizes. We propose that they might serve as a model for pediatric heart valve reconstruction and replacement valve design. We explore this concept using computer simulation.MethodsWe use a finite element method to simulate the ability of a reconstructed cardiac semilunar valve to close competently in a growing vessel, comparing a 3-leaflet design with a 2-leaflet design that mimics a venous valve. Three venous valve designs were simulated to begin to explore the parameter space.ResultsSimulations show that for an initial vessel diameter of 12 mm, the venous valve design remains competent as the vessel grows to 20 mm (67%), whereas the normal semilunar design remains competent only to 13 mm (8%). Simulations also suggested that systolic function, estimated as effective orifice area, was not detrimentally affected by the venous valve design, with all 3 venous valve designs exhibiting greater effective orifice area than the semilunar valve design at a given level of vessel growth.ConclusionsMorphologic features of the venous valve design make it well suited for competent closure over a wide range of vessel sizes, suggesting its use as a model for semilunar valve reconstruction in the growing child.
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Project description:22q11.2 deletion syndrome is mainly characterized by conotruncal congenital heart defects, velopharyngeal insufficiency, hypocalcemia and a characteristic craniofacial appearance. The etiology in the majority of patients is a 3-Mb recurrent deletion in region 22q11.2. Nevertheless, recently some cases of infrequent deletions with various sizes have been reported with a different phenotype. We report on a patient with congenital heart disease (truncus arteriosus type 2) in whom a de novo 1.3-Mb 22q11.2 deletion was detected by array comparative genomic hybridization. The deletion described corresponds to an atypical and distal deletion which spans low copy repeat (LCR) 4 and is associated with breakpoint sites that do not correspond to known LCRs of 22q11.2. We examine the clinical phenotype of our case and compare our findings with those published in the literature. The most prevalent clinical features in this type of deletion are a history of prematurity, pre-natal and post-natal growth retardation, slight facial dysmorphic features, microcephaly and developmental delay, with a speech defect in particular. These are clearly different from those found in the classic 22q11.2 deletion syndrome, and we believe that the main differential diagnosis should be with Silver-Russel syndrome. In our case we observe the cardiac phenotype with truncus arteriosus communis usually seen in the classic 22q11.2 deletion syndrome, and so far associated with the TBX1 gene. Significantly, however, TBX1 is not included in our patient's deletion. The possible roles of a position effect or other genes are discussed.
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Project description:Background: The outflow tract of crocodilians resembles that of birds and mammals as ventricular septation is complete. The arterial anatomy, however, presents with a pulmonary trunk originating from the right ventricular cavum, and two aortas originating from either the right or left ventricular cavity. Mixing of blood in crocodilians cannot occur at the ventricular level as in other reptiles but instead takes place at the aortic root level by a shunt, the foramen of Panizza, the opening of which is guarded by two facing semilunar leaflets of both bicuspid aortic valves. Methods: Developmental stages of Alligator mississipiensis, Crocodilus niloticus and Caiman latirostris were studied histologically. Results and Conclusions: The outflow tract septation complex can be divided into two components. The aorto-pulmonary septum divides the pulmonary trunk from both aortas, whereas the interaortic septum divides the systemic from the visceral aorta. Neural crest cells are most likely involved in the formation of both components. Remodeling of the endocardial cushions and both septa results in the formation of bicuspid valves in all three arterial trunks. The foramen of Panizza originates intracardially as a channel in the septal endocardial cushion.
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Project description:Cxcr7-/- mice die a few hours after birth. All of them display semilunar valves abnormalities, including bicuspid aortic or pulmonary valves. Those defects only become obvious before birth. Keywords: genetic modification
2007-11-01 | GSE8710 | GEO