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

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

Heart ◽

10.1136/heartjnl-2017-312452 ◽

2018 ◽

Vol 104 (14) ◽

pp. 1200-1207 ◽

Cited By ~ 6

Author(s):

Gijsbert F L Kapel ◽

Sergio Laranjo ◽

Nico A Blom ◽

Mark G Hazekamp ◽

Martin J Schalij ◽

...

Keyword(s):

Ventricular Tachycardia ◽

Ventricular Septal Defect ◽

Tetralogy Of Fallot ◽

Patch Size ◽

Septal Defect ◽

Pulmonary Valve ◽

Strongly Correlated ◽

Tricuspid Annulus ◽

Transannular Patch ◽

Life Mode

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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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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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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Oxygen uptake transient kinetics during constant-load exercise in children after operations of ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, or tricuspid valve atresia

The American Journal of Cardiology ◽

10.1016/0002-9149(94)90091-4 ◽

1994 ◽

Vol 74 (2) ◽

pp. 166-169 ◽

Cited By ~ 8

Author(s):

Peter Gildein ◽

Rolf Mocellin ◽

Klaus Kaufmehl

Keyword(s):

Ventricular Septal Defect ◽

Oxygen Uptake ◽

Tetralogy Of Fallot ◽

Tricuspid Valve ◽

Septal Defect ◽

Constant Load ◽

Transient Kinetics ◽

Constant Load Exercise

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Management of Patients following Correction of Tetralogy of Fallot and Ventricular Septal Defect

CHEST Journal ◽

10.1378/chest.86.4.652-a ◽

1984 ◽

Vol 86 (4) ◽

pp. 652

Author(s):

Maria Serratto

Keyword(s):

Ventricular Septal Defect ◽

Tetralogy Of Fallot ◽

Septal Defect

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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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AN UNUSUAL CASE OF BIVENTRICULAR HYPERTROPHY: A RARE ADULTHOOD DIAGNOSIS

10.22541/au.163587875.58120613/v1 ◽

2021 ◽

Author(s):

Rosa Lillo ◽

Angelica Bibiana Delogu ◽

Gessica Ingrasciotta ◽

Gianluigi Perri ◽

Maria Grandinetti ◽

...

Keyword(s):

Chest Pain ◽

Hypertrophic Cardiomyopathy ◽

Ventricular Septal Defect ◽

Unusual Case ◽

Septal Defect ◽

Congenital Abnormalities ◽

Multimodality Imaging ◽

Final Diagnosis ◽

Imaging Evaluation ◽

Anomalous Pulmonary Vein

A woman complaining of dyspnea and chest pain since childhood, was referred to our hospital with an initial diagnosis of biventricular hypertrophic cardiomyopathy. Multimodality imaging evaluation revealed massive right ventricular (RV) hypertrophy and severe RV outflow tract obstruction, with a final diagnosis of double chambered RV associated with small ventricular septal defect with right-to-left shunt and right partial anomalous pulmonary vein return. This represents an uncommon combination of congenital abnormalities, extremely rarely diagnosed in adulthood.

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