Impact of surgery on presence and dimensions of anatomical isthmuses in tetralogy of Fallot

ObjectiveIn tetralogy of Fallot (TOF), the dominant ventricular tachycardia substrates are slow-conducting anatomical isthmuses. Surgical correction has evolved, which might have influenced isthmus presence and dimensions.MethodsOne hundred and forty-two postmortem TOF specimens (84/58 corrected/uncorrected) were studied for isthmus presence. Isthmus 1 is located between the tricuspid annulus and right ventricular (RV) outflow tract (RVOT) patch/RV incision, isthmus 2 between RVOT patch/RV incision and pulmonary valve, isthmus 3 between pulmonary valve and ventricular septal defect (patch), isthmus 4 between ventricular septal defect (patch) and tricuspid annulus. Isthmus width and thickness were measured.ResultsOf 84 corrected postmortem TOF specimens (death: 6.6 years (4.0–11.5)), 83 demonstrated isthmus 1 (99%, width=25±10 mm, thickness=5±2 mm), 35 isthmus 2 (42%, width=10±9 mm, thickness=3±2 mm), 83 isthmus 3 (99%, width=10±6 mm, thickness=5±2 mm), and 5 isthmus 4 (6%, width=4±2 mm, thickness=2±1 mm). Transatrial-transpulmonary correction (n=49) as compared with transventricular correction (n=35) prevented isthmus 2 (0% vs 100%, P<0.001). Transatrial-transpulmonary correction at age <1 year (n=7) as compared with ≥1 year (n=42) required a smaller transannular RVOT patch (28±15 vs 45±14 mm, P<0.001). Mode and timing of correction did not influence presence and dimensions of isthmus 3. In corrected and uncorrected TOF specimens (death 1.8 years (0.5–6.6)), the range of isthmus 3 dimensions was broad (width: min=2 mm, max=32 mm; thickness: min=1, max 13 mm) across all ages. Isthmus 3 width and thickness were strongly correlated (r=0.65, P<0.001).ConclusionsIn TOF, the current routine use of transatrial-transpulmonary correction prevents isthmus 2. Correction <1 year reduces transannular patch size, which may influence isthmus 1 width later in life. Mode and timing of correction did not change prevalence and dimensions of isthmus 3, in which dimensions varied widely in uncorrected and corrected TOF.

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Echo/Doppler diagnosis of tetralogy of Fallot, ventricular septal defect, pulmonary valve dysplasia, and hypertrophic cardiomyopathy in WKY/NCrj rats.

Japanese Circulation Journal ◽

10.1253/jcj.56.441 ◽

1992 ◽

Vol 56 (5) ◽

pp. 441-451 ◽

Cited By ~ 2

Author(s):

TAKASHI NAKAMURA ◽

TOSHIRO KURIBAYASHI ◽

KAZUTOSHI SHIMOO ◽

HIROSHI KATSUME ◽

MASAO NAKAGAWA ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Ventricular Septal Defect ◽

Tetralogy Of Fallot ◽

Septal Defect ◽

Pulmonary Valve

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Tetralogy of Fallot with Absent Pulmonary Valve - A Case Report

University Heart Journal ◽

10.3329/uhj.v6i1.7197 ◽

1970 ◽

Vol 6 (1) ◽

pp. 48-50

Author(s):

Md Mazibur Rahman

Keyword(s):

Heart Failure ◽

Tetralogy Of Fallot ◽

Septal Defect ◽

Pulmonary Valve ◽

Right Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Absent Pulmonary Valve ◽

Transannular Patch ◽

Patch Closure ◽

Successful Repair

A 25 years male patient of Tetralogy of Fallot (TOF) with congenital absent of pulmonary valve (APV) presented with symptoms of palpitation and exertional respiratory distress without congestive heart failure. He underwent successful repair of intracardiac defects. The procedures consisted of patch closure of ventricular septal defect and right. ventricular outflow tract reconstruction with a monocusp transannular patch. Resection or plication of dilated pulmonary artery was not required. The patient is doing well without any symptoms. Key words: Tetralogy of Fallot; Absent pulmonary valve DOI: 10.3329/uhj.v6i1.7197University Heart Journal Vol.6(1) 2010 pp.48-50

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Aortic Root Translocation for Repair of Transposition of Great Arteries With Absent Pulmonary Valve

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135118820865 ◽

2019 ◽

Vol 10 (4) ◽

pp. 513-514

Author(s):

Ashish Katewa ◽

Nishith Bhargava ◽

Balswaroop Sahu ◽

Atul Prabhu ◽

Shivani Gajpal

Keyword(s):

Ventricular Septal Defect ◽

Tetralogy Of Fallot ◽

Congenital Heart Defect ◽

Aortic Root ◽

Rare Case ◽

Congenital Heart ◽

Septal Defect ◽

Pulmonary Valve ◽

Absent Pulmonary Valve ◽

Transposition Of Great Arteries

Absent pulmonary valve is a rare congenital heart defect where the pulmonary valve is either absent or rudimentary. It is usually associated with tetralogy of Fallot although it has been reported with other lesions. We present a rare case of absent pulmonary valve associated with transposition of great arteries and non-routable ventricular septal defect that was surgically managed by aortic root translocation.

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Double-chambered right ventricle masquerading as bipartite right ventricle

Cardiology in the Young ◽

10.1017/s1047951121002572 ◽

2021 ◽

pp. 1-2

Author(s):

A. Shaheer Ahmed ◽

Tushar Agarwal

Keyword(s):

Left Ventricle ◽

Ventricular Septal Defect ◽

Right Ventricle ◽

Septal Defect ◽

Pulmonary Valve ◽

Tricuspid Annulus ◽

Pulmonary Consolidation ◽

Depth Analysis ◽

The Right ◽

Rule Out

Abstract A 10-day-old neonate with pulmonary consolidation was referred for echocardiography to rule out CHD. At first glance, the morphology appeared to be a bipartite right ventricle with normal tricuspid and pulmonary valves. In-depth analysis, however, of the images showed a double-chambered right ventricle, in which the inlet and outlet portions of the right ventricle were isolated from the apical component of the right ventricle, which itself communicated with the left ventricle through a ventricular septal defect. There was a normal pulmonary valve and tricuspid annulus.

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Right-sided abnormalities

10.1093/med/9780198766520.003.0011 ◽

2018 ◽

Author(s):

Nick Archer ◽

Nicky Manning

Keyword(s):

Ventricular Septal Defect ◽

Tetralogy Of Fallot ◽

Septal Defect ◽

Pulmonary Valve ◽

Pulmonary Stenosis ◽

Pulmonary Atresia ◽

Venous Drainage ◽

Double Outlet Right Ventricle ◽

Absent Pulmonary Valve ◽

Atrioventricular Junction

This chapter explores right-sided abnormalities, discussing the venoatrial junction (including both azygous/hemiazygous connections and abnormal systemic venous drainage), the atrioventricular junction (including tricuspid atresia and Ebstein’s anomaly/tricuspid valve dysplasia), and the ventriculoarterial junction (including pulmonary stenosis, pulmonary atresia with intact septum, tetralogy of Fallot, tetralogy of Fallot with absent pulmonary valve, pulmonary atresia with ventricular septal defect, and double outlet right ventricle), and arterial abnormalities.

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Tetralogy of Fallot, Pulmonary Valve Stenosis, Ventricular Septal Defect, and Hypertrophic Cardiomyopathy in WKY/NCrj Rats

Pediatric Research ◽

10.1203/00006450-199005000-00013 ◽

1990 ◽

Vol 27 (5) ◽

pp. 483-487 ◽

Cited By ~ 6

Author(s):

Toshiro Kuribayashj ◽

Kazutoshi Shimoo ◽

Takashi Nakamura ◽

Hirofumi Taniwaki ◽

Kenji Hamaoka ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Ventricular Septal Defect ◽

Tetralogy Of Fallot ◽

Septal Defect ◽

Pulmonary Valve ◽

Pulmonary Valve Stenosis

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Clinical presentation, natural history, and outcome of patients with the absent pulmonary valve syndrome

Cardiology in the Young ◽

10.1017/s1047951104004093 ◽

2004 ◽

Vol 14 (4) ◽

pp. 402-408 ◽

Cited By ~ 35

Author(s):

Nili Zucker ◽

Ilya Rozin ◽

Aviva Levitas ◽

Eli Zalzstein

Keyword(s):

Ventricular Septal Defect ◽

Tetralogy Of Fallot ◽

Septal Defect ◽

Clinical Presentation ◽

Pulmonary Valve ◽

Ventricular Septum ◽

Intact Ventricular Septum ◽

Absent Pulmonary Valve ◽

History Of ◽

Absent Pulmonary Valve Syndrome

So-called ‘absent pulmonary valve syndrome’ is a rare cardiac malformation, usually associated with tetralogy of Fallot. Congenital absence of the leaflets of the pulmonary valve is less common when the ventricular septum is intact. Characteristic features of the syndrome include dysplasia or absence of the pulmonary valvar leaflets, permitting severe pulmonary regurgitation, and aneurysmal dilation of the pulmonary arteries. The purpose of our study was to review our experience with patients diagnosed as having the absent pulmonary valve syndrome, and to describe their clinical presentation, natural history, and outcome. We reviewed retrospectively data from 18 patients with absent pulmonary valve syndrome, 10 boys and eight girls, treated between March 1983 and May 2003. We identified two groups of patients, one made up of 11 patients with a ventricular septal defect, in whom the morphology of the subpulmonary outflow tract was phenotypic for tetralogy of Fallot, and another group, with seven patients, having an intact ventricular septum. Family history of congenital heart disease was common only in patients with ventricular septal defect, being found in 73%, all of whom were diagnosed during infancy with variable respiratory distress. Diagnosis was delayed in 43% of the patients with an intact ventricular septum. Cardiac surgery was performed in eight patients with ventricular septal defect (73%), compared to only two patients (28%) with an intact ventricular septum. Overall mortality was 28%, with five patients dying. Although our sample was small, two clinical patterns emerged depending on the presence or absence of a ventricular septal defect. Patients with a ventricular septal defect and phenotypic features of tetralogy of Fallot have a strong family history of congenital cardiac disease, develop respiratory symptoms during infancy and exhibit a variable prognosis, despite cardiac surgery. Patients with an intact ventricular septum are usually asymptomatic, present later in life, and show a relatively benign prognosis.

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