scholarly journals The right ventricle, outflow tract, and ventricular septum comprise a restricted expression domain within the secondary/anterior heart field

2005 ◽  
Vol 287 (1) ◽  
pp. 134-145 ◽  
Author(s):  
Michael P. Verzi ◽  
David J. McCulley ◽  
Sarah De Val ◽  
Evdokia Dodou ◽  
Brian L. Black
1991 ◽  
Vol 1 (2) ◽  
pp. 105-113 ◽  
Author(s):  
Robert H. Anderson ◽  
Christine Anderson ◽  
James R. Zuberbuhler

SummaryAtresia of the outflow tract of the right ventricle in the presence of an intact ventricular septum poses major problems for surgical management. In the light of known problems, 43 autopsied hearts with this congenital abnormality were evaluated to note the arrangement of the myocardium and the cavity of the right ventricle, the state of the tricuspid valve, the precise substrate for pulmonary atresia, and the presence offistulous communications between the ventricular cavity and the coronary arteries. The specimens could be divided into two groups. The first group (38 hearts) had hypoplasia of the cavity of the right ventricle with mural hypertrophy while the second group (5 hearts) had a dilated right ventricular cavity with thinning of the wall. The larger group could be divided into two subgroups on the basis of the substrate for pulmonary atresia. Twenty hearts had muscular atresia of the right ventricular outflow tract and 18 had a potentially patent outflow tract blocked by an imperforate pulmonary valve. A comparison of these two subgroups revealed that mural hypertrophy and cavitary hypoplasia were more severe in hearts with muscular atresia. The tricuspid valve was hypoplastic and its leaflets were not dysplastic in this group but the leaflets were dysplastic in hearts with valvar pulmonary atresia. Fistulous communications between the ventricular cavity and the coronary arteries were only seen in the cases with muscular atresia. In this series, recognition of muscular pulmonary atresia would have identified those patients with the worst surgical prognosis.


2018 ◽  
Author(s):  
Jielin Liu ◽  
Henghui Cheng ◽  
Menglan Xiang ◽  
Lun Zhou ◽  
Ke Zhang ◽  
...  

AbstractDominant mutations of Gata4, an essential cardiogenic transcription factor (TF), cause outflow tract (OFT) defects in both human and mouse. We investigated the molecular mechanism underlying this requirement. Gata4 happloinsufficiency in mice caused OFT defects including double outlet right ventricle (DORV) and conal ventricular septum defects (VSDs). We found that Gata4 is required within Hedgehog (Hh)-receiving second heart field (SHF) progenitors for normal OFT alignment. Increased Pten-mediated cell-cycle transition, rescued atrial septal defects but not OFT defects in Gata4 heterozygotes. SHF Hh-receiving cells failed to migrate properly into the proximal OFT cushion in Gata4 heterozygote embryos. We find that Hh signaling and Gata4 genetically interact for OFT development. Gata4 and Smo double heterozygotes displayed more severe OFT abnormalities including persistent truncus arteriosus (PTA) whereas restoration of Hedgehog signaling rescued OFT defects in Gata4-mutant mice. In addition, enhanced expression of the Gata6 was observed in the SHF of the Gata4 heterozygotes. These results suggested a SHF regulatory network comprising of Gata4, Gata6 and Hh-signaling for OFT development. This study indicates that Gata4 potentiation of Hh signaling is a general feature of Gata4-mediated cardiac morphogenesis and provides a model for the molecular basis of CHD caused by dominant transcription factor mutations.Author SummaryGata4 is an important protein that controls the development of the heart. Human who possess a single copy of Gata4 mutation display congenital heart defects (CHD), including the double outlet right ventricle (DORV). DORV is an alignment problem in which both the Aorta and Pulmonary Artery originate from the right ventricle, instead of originating from the left and the right ventricles, respectively. To study how Gata4 mutation causes DORV, we used a Gata4 mutant mouse model, which displays DORV. We showed that Gata4 is required in the cardiac precursor cells for the normal alignment of the great arteries. Although Gata4 mutation inhibits the rapid increase in number of the cardiac precursor cells, rescuing this defects does not recover the normal alignment of the great arteries. In addition, there is a movement problem of the cardiac precursor cells when migrating toward the great arteries during development. We further showed that a specific molecular signaling, Hh-signaling, is responsible to the Gata4 action in the cardiac precursor cells. Importantly, over-activating the Hh-signaling rescues the DORV in the Gata4 mutant embryos. This study provides an explanation for the ontogeny of CHD.


1992 ◽  
Vol 2 (4) ◽  
pp. 391-394 ◽  
Author(s):  
Carlo Vosa ◽  
Paolo Arciprete ◽  
Giuseppe Caianiello ◽  
Gaetano Palma

SummaryBetween February 1986 and December 1991, 41 patients with pulmonary atresia and intact ventricular septum were treated in our institution following a multistage protocol of management. In all cases, the first step was to construct a right modified Blalock-Taussig shunt during the neonatal period regardless of the right ventricular anatomy. Then, in patients with well-developed right ventricles possessing all three components, we proceeded to early surgical repair. In contrast, in patients with right ventricles having obliteration of some components, yet deemed to be recoverable, the next step was to provide palliative relief of obstruction in the right ventricular outflow tract followed, if possible, by subsequent repair. Fontan's operation was performed in patients with right ventricles considered unsuitable from the outset to support the pulmonary circulation. Only one patient died following the initial shunt procedure (mortality of 2.43%). The subsequent program of treatment has now been concluded in 22 patients. In all those deemed to have favorable native anatomy (10 cases), the subsequent complete repair was successful. Among the 24 patients who required palliation of the outflow tract, five died while total repair was subsequently performed in eight. Fontan's operation was performed without mortality in five patients with small right ventricles, although one patient died while waiting for surgery. In all, 89 procedures were performed with an overall mortality of 14%.


1991 ◽  
Vol 101 (2) ◽  
pp. 222-229 ◽  
Author(s):  
William G. Williams ◽  
Patricia Burrows ◽  
Robert M. Freedom ◽  
George A. Trusler ◽  
John G. Coles ◽  
...  

1996 ◽  
Vol 199 (2) ◽  
pp. 359-365 ◽  
Author(s):  
M Axelsson ◽  
C E Franklin ◽  
C O Löfman ◽  
S Nilsson ◽  
G C Grigg

Prolonged submergence imposes special demands on the cardiovascular system. Unlike the situation in diving birds and mammals, crocodilians have the ability to shunt blood away from the lungs, despite having an anatomically divided ventricle. This remarkable cardiovascular flexibility is due in part to three anatomical peculiarities: (1) an 'extra' aorta (the left aorta) that leaves the right ventricle and allows the blood from the right ventricle to take an alternative route into the systemic circulation instead of going to the lungs; (2) the foramen of Panizza, an aperture that connects the right and left aortas at their base immediately outside the ventricle; and (3) a set of connective tissue outpushings in the pulmonary outflow tract in the right ventricle. Using high-resolution angioscopy, we have studied these structures in the beating crocodile heart and correlated their movements with in vivo pressure and flow recordings. The connective tissue outpushings in the pulmonary outflow tract represent an active mechanism used to restrict blood flow into the lungs, thus creating one of the conditions required for a right-to-left shunt. We observed that the foramen of Panizza was obstructed by the medial cusp of the right aortic valve during most of systole, effectively differentiating the left and right aortic blood pressure. During diastole, however, the foramen remained open, allowing pressure equilibration between the two aortas. Contrary to current theories, we found that the left aortic valves were unable to cover the foramen of Panizza during any part of the cardiac cycle, supporting the reversed foramen flow hypothesis. This would ensure a supply of blood to the coronary and cephalic circulation during a complete shut-down of the left side of the heart, such as might occur during prolonged submergence.


2013 ◽  
Vol 95 (5) ◽  
pp. 1670-1674 ◽  
Author(s):  
Qian-zhen Li ◽  
Hua Cao ◽  
Qiang Chen ◽  
Gui-Can Zhang ◽  
Liang-Wan Chen ◽  
...  

1991 ◽  
Vol 260 (4) ◽  
pp. H1087-H1097
Author(s):  
J. E. Calvin

The purpose of this study was to determine whether segment lengths measured from the right ventricular inflow and outflow tract regions of the right ventricle would accurately reflect true volume changes of the right ventricle and to determine the response of the right ventricle to afterload increases induced by both constricting the pulmonary artery (PAC) and embolizing the pulmonary circulation with glass beads (GBE). Three excised hearts were instrumented with segment-length crystals attached to the inflow and outflow tract regions, and saline was instilled into a balloon implanted inside the right ventricular cavity. The experiments showed a high correlation (r greater than or equal to 0.90 in all cases) between static segment lengths and volume instilled. In open chest, open pericardial canine experiments, vena caval occlusion reduced end-diastolic segments lengths and right ventricular systolic pressure consistent with a reduction in right ventricular end-diastolic volume. In a separate group of animals, volume loading with dextran increased inflow and outflow end-diastolic segment lengths and increased cardiac output. In two further groups of animals, one of which was pretreated intravenously with propranolol (Inderal), both forms of pressure overload increased end-diastolic lengths in both regions. However, GBE increased right ventricular stroke work compared with PAC. We conclude that end-diastolic segment lengths reflect true volume changes of the right ventricle. Furthermore, during acute pressure overload, the right ventricle dilates to compensate for the afterload change. However, ventricular function is better maintained after GBE.


2019 ◽  
Vol 30 (1) ◽  
pp. 126-128
Author(s):  
Cheul Lee ◽  
Kyung Min Kim ◽  
Jae Young Lee ◽  
Jihong Yoon

AbstractTricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.


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