Sectional anatomy of the ventricular septal defect in double outlet right ventricle—correlation of magnetic resonance images from autopsied hearts with anatomic sections

AbstractA ventricular septal defect is, almost always, an integral part of double outlet right ventricle and has been classified into the subaortic, subpulmonary, doubly committed and non-committed varieties. This study was performed to correlate the cross-sectional imaging characteristics of such ventricular septal defect in double outlet right ventricles using pathological specimens. The extent and the orientation of the outlet septum were the most important in the differentiation of the four varieties of ventricular septal defect. In the subaortic variety, the outlet septum fused with the left anterior margin of the defect, this being marked by the anterior limb of the septomarginal trabeculation. In the subpulmonary variety, the outlet septum fused with the right posterior margin of the defect, this being the posterior limb of the septomarginal trabeculation. The outlet septum was vestigial in case with doubly committed defects. In those with non-committed defects, the defect was not shown in those images or sections which demonstrated the outlet septum.

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Tetralogy of Fallot: nosological, morphological, and morphogenetic considerations

Cardiology in the Young ◽

10.1017/s1047951113001686 ◽

2013 ◽

Vol 23 (6) ◽

pp. 858-866 ◽

Cited By ~ 15

Author(s):

Robert H. Anderson ◽

Diane E. Spicer ◽

Jorge M. Giroud ◽

Timothy J. Mohun

Keyword(s):

Ventricular Septal Defect ◽

Right Ventricle ◽

Tetralogy Of Fallot ◽

Septal Defect ◽

Morphological Changes ◽

Double Outlet Right Ventricle ◽

125Th Anniversary ◽

Initial Description ◽

The Right ◽

Subpulmonary Stenosis

AbstractIt is timely, in the 125th anniversary of the initial description by Fallot of the hearts most frequently seen in patients presenting with “la maladie bleu”, that we revisit his descriptions, and discuss his findings in the light of ongoing controversies. Fallot described three hearts in his initial publication, and pointed to the same tetralogy of morphological features that we recognise today, namely, an interventricular communication, biventricular connection of the aorta, subpulmonary stenosis, and right ventricular hypertrophy. In one of the hearts, he noted that the aorta arose exclusively from the right ventricle. In other words, one of his initial cases exhibited double-outlet right ventricle. When we now compare findings in hearts with the features of the tetralogy, we can observe significant variations in the nature of the borders of the plane of deficient ventricular septation when viewed from the aspect of the right ventricle. We also find that this plane, usually described as the ventricular septal defect, is not the same as the geometric plane separating the cavities of the right and left ventricles. This means that the latter plane, the interventricular communication, is not necessarily the same as the ventricular septal defect. We are now able to provide further insights into these features by examining hearts prepared from developing mice. Additional molecular investigations will be required, however, to uncover the mechanisms responsible for producing the morphological changes underscoring tetralogy of Fallot.

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Repair of double outlet right ventricle with doubly-committed ventricular septal defect

Cardiology in the Young ◽

10.1017/s1047951101000531 ◽

2001 ◽

Vol 11 (4) ◽

pp. 415-419 ◽

Cited By ~ 6

Author(s):

Hideki Uemura ◽

Toshikatsu Yagihara ◽

Takayuki Kadohama ◽

Youichi Kawahira ◽

Yoshiro Yoshikawa

Keyword(s):

Ventricular Septal Defect ◽

Right Ventricle ◽

Septal Defect ◽

Pulmonary Stenosis ◽

Pulmonary Arteries ◽

Ventricular Outflow Tract ◽

Double Outlet Right Ventricle ◽

Left Ventricular ◽

Aortic Orifice ◽

The Right

Objective: To investigate our surgical results of intraventricular rerouting in patients having double outlet right ventricle with doubly-committed ventricular septal defect. Methods: We undertook repair in 8 patients with this particular feature. Of these, 2 patients had pulmonary stenosis, and another had interruption of the aortic arch. The subarterial defect was unequivocally related to both the aortic and the pulmonary orifices in all, albeit slightly deviated towards the aortic orifice in one, and towards the pulmonary orifice in another. Intraventricular rerouting was carried out via incisions to the right atrium and the pulmonary trunk. To ensure reconstruction of an unobstructed pulmonary pathway, a limited right ventriculotomy was made in 5. Results: All patients survived the procedure, and are currently doing well, with follow-up of 25 to 194 months, with a mean of 117 ± 68 months. Catheterization carried out 16 ± 6 months after repair demonstrated excellent ventricular parameters. Mean pulmonary arterial pressure was 16 ± 7 mmHg, being higher than 20 mmHg in 2 patients. No significant obstruction was found between the right ventricle and the pulmonary arteries. A pressure gradient across the left ventricular outflow tract became significant in one patient in whom a small outlet septum was present, and a heart-shaped baffle had been used for intraventricular rerouting. Reoperation was eventually needed in this patient for treatment of the obstruction, which proved to be progressive. Conclusion: Precise recognition of the morphologic features is of paramount importance when choosing the optimal options for biventricular repair in patients with double outlet right ventricle and doubly-committed interventricular communication.

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Double-Outlet Right Ventricle with Transposed Great Arteries and Subpulmonary Ventricular Septal Defect

Circulation ◽

10.1161/01.cir.39.5s1.i-207 ◽

1969 ◽

Vol 39 (5s1) ◽

Cited By ~ 9

Author(s):

BILLY M. HIGHTOWER ◽

ALBERTO BARCIA ◽

LIONEL M. BARGERON ◽

JOHN W. KIRKLIN

Keyword(s):

Ventricular Septal Defect ◽

Right Ventricle ◽

Septal Defect ◽

Double Outlet Right Ventricle

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Double-Outlet Right Ventricle in a Chianina Calf

Animals ◽

10.3390/ani11020318 ◽

2021 ◽

Vol 11 (2) ◽

pp. 318

Author(s):

Domenico Caivano ◽

Maria Chiara Marchesi ◽

Piero Boni ◽

Noemi Venanzi ◽

Giovanni Angeli ◽

...

Keyword(s):

Right Ventricle ◽

Septal Defect ◽

Heart Defects ◽

Double Outlet Right Ventricle ◽

Cardiac Malformation ◽

Septal Defects ◽

Congenital Cardiac Malformation ◽

The Right ◽

Similar Complex ◽

Echocardiographic Findings

Congenital heart defects have been occasionally reported in cattle and ventricular septal defect represents the most frequently encountered anomaly. The double-outlet right ventricle is a rare congenital ventriculoarterial malformation reported only in certain cattle breeds. We describe this rare and complex congenital cardiac malformation observed in a 10-day-old male Chianina calf. Clinical examination showed tachycardia, tachypnea, jugular pulses, cyanotic mucous membranes and a right apical systolic murmur. Transthoracic echocardiography revealed severe dilation of the right-sided cardiac chambers with a markedly hypoplastic left ventricle. Both aorta and pulmonary artery leaving the right ventricle in parallel alignment with the tricuspid valve were suggestive of a dual-outlet right ventricle. Interventricular and interatrial septal defects were also visualized. Post-mortem examination confirmed the echocardiographic findings. To the authors’ knowledge, a similar complex congenital cardiac malformation has not been reported in calves of the Chianina breed to date.

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Ventricular non-compaction in the setting of double-outlet right ventricle (tetralogy of Fallot type) with doubly committed subarterial ventricular septal defect

Heart ◽

10.1136/hrt.2006.090480 ◽

2007 ◽

Vol 93 (5) ◽

pp. 647-647 ◽

Cited By ~ 2

Author(s):

D. Kenny ◽

D. Hares ◽

O. Uzun

Keyword(s):

Ventricular Septal Defect ◽

Right Ventricle ◽

Tetralogy Of Fallot ◽

Septal Defect ◽

Double Outlet Right Ventricle

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Double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly) and other complex congenital cardiac malformations in an American Quarter Horse foal

Journal of Veterinary Cardiology ◽

10.1016/j.jvc.2017.10.005 ◽

2018 ◽

Vol 20 (1) ◽

pp. 64-72 ◽

Cited By ~ 4

Author(s):

Rebecca Kohnken ◽

Karsten Schober ◽

Jennifer Godman ◽

Alison Gardner ◽

Tiffany Jenkins ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Right Ventricle ◽

Septal Defect ◽

Double Outlet Right Ventricle ◽

Cardiac Malformations ◽

Quarter Horse

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The guide wire was gently advanced through the ventricular septal defect (VSD) into the right ventricle

ASVIDE ◽

10.21037/asvide.2018.778 ◽

2018 ◽

Vol 5 ◽

pp. 778-778

Author(s):

Shouzheng Wang ◽

Wenbin Ouyang ◽

Yao Liu ◽

Fengwen Zhang ◽

Gaili Guo ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Right Ventricle ◽

Septal Defect ◽

Guide Wire ◽

The Right

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Early and late outcomes of surgical repair of double-chambered right ventricle: a single-centre experience

Cardiology in the Young ◽

10.1017/s1047951120000244 ◽

2020 ◽

Vol 30 (3) ◽

pp. 409-412

Author(s):

Murat Surucu ◽

İlkay Erdoğan ◽

Birgül Varan ◽

Murat Özkan ◽

N. Kürşad Tokel ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Right Ventricle ◽

Septal Defect ◽

Surgical Intervention ◽

Systolic Pressure ◽

Presenting Symptoms ◽

Long Term Follow Up ◽

The Right

AbstractObjective:Double-chambered right ventricle is characterised by division of the outlet portion of the right ventricle by hypertrophy of the septoparietal trabeculations into two parts. We aim to report our experiences regarding the presenting symptoms of double-chambered right ventricle, long-term prognosis, including the recurrence rate and incidence of arrhythmias after surgery.Methods:We retrospectively investigated 89 consecutive patients who were diagnosed to have double-chambered right ventricle and underwent a surgical intervention from 1995 to 2016. The data obtained by echocardiography, cardiac catheterisation, and surgical findings as well as post-operative follow-up, surgical approaches, post-operative morbidity, mortality, and cardiac events were evaluated.Results:Median age at the time of diagnosis was 2 months and mean age at the time of operation was 5.3 years. Concomitant cardiac anomalies were as follows: perimembranous ventricular septal defect (78 patients), atrial septal defect (9 patients), discrete subaortic membrane (32 patients), right aortic arch (3 patients), aortic valve prolapse and/or mild aortic regurgitation (14 patients), and left superior caval vein (2 patients). The mean follow-up period was 4.86 ± 4.6 years. In these patients, mean systolic pressure gradient in the right ventricle by echocardiography before, immediately, and long-term after surgical intervention was 66.3, 11.8, and 10.4 mmHg, respectively. There were no deaths during the long-term follow-up period. Surgical reinterventions were performed for residual ventricular septal defect (2), residual pulmonary stenosis (1), and severe tricuspid insufficiency (1).Conclusion:The surgical outcomes and prognosis of double-chambered right ventricle are favourable, recurrence and fatal arrhythmias are unlikely in long-term follow-up.

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Failure of Cellularization of Ventriculotomy Patch Leading to Right Ventricular Pseudoaneurysm

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135119880547 ◽

2019 ◽

Vol 11 (1) ◽

pp. 123-126

Author(s):

Sruti Rao ◽

Robert D. Stewart ◽

Gosta Pettersson ◽

Carmela Tan ◽

Suzanne Golz ◽

...

Keyword(s):

Left Ventricle ◽

Ventricular Septal Defect ◽

Right Ventricle ◽

Right Ventricular ◽

Septal Defect ◽

Tricuspid Atresia ◽

Outflow Obstruction ◽

Ventricular Outflow Obstruction ◽

The Right ◽

Double Inlet Left Ventricle

Enlargement of the bulboventricular foramen (BVF) in double-inlet left ventricle or the ventricular septal defect (VSD) in tricuspid atresia with transposition of the great arteries is one approach for prevention or treatment of systemic ventricular outflow obstruction. Most often, BVF/VSD restriction is bypassed preemptively or addressed directly at the time of Glenn/Fontan procedures as part of staged univentricular palliation. We describe a patient who underwent enlargement of a restrictive VSD during Fontan completion and subsequently presented with an asymptomatic pseudoaneurysm of the right ventricle at the ventriculotomy site.

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