scholarly journals Transventricular valvotomy for pulmonary atresia with intact ventricular septum in neonates: a single-centre experience in mid-term follow-up

2014 ◽  
Vol 47 (1) ◽  
pp. 168-172 ◽  
Author(s):  
Renjie Hu ◽  
Haibo Zhang ◽  
Wei Dong ◽  
Xinrong Liu ◽  
Zhiwei Xu ◽  
...  
Keyword(s):  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Single Centre ◽  
Single Centre Experience

Outcomes of Radiofrequency Perforation for Pulmonary Atresia and Intact Ventricular Septum: A Single-Centre Experience

2016 ◽  
Vol 38 (1) ◽  
pp. 170-175 ◽  
Author(s):  
Steven Rathgeber ◽  
Benjamin Auld ◽  
Stephanie Duncombe ◽  
Martin C. K. Hosking ◽  
Kevin C. Harris
Keyword(s):  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Single Centre ◽  
Single Centre Experience ◽  
Radiofrequency Perforation

scholarly journals 680 Pulmonary Atresia With Intact Ventricular Septum – A Single Centre Experience

2020 ◽  
Vol 29 ◽  
pp. S342-S343
Author(s):  
K. Hockey ◽  
L. Yeo ◽  
M. Liava'a ◽  
P. Roberts ◽  
C. Lawley
Keyword(s):  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Single Centre ◽  
Single Centre Experience

scholarly journals Successful multiple Percutaneous Interventions and Biventricular Surgical Repair in a Child with Pulmonary Atresia with Intact Ventricular Septum— A Case Report

2020 ◽  
Vol 13 (1) ◽  
pp. 81-85
Author(s):  
Nurun Nahar Fatema Begum ◽  
Md Ferdousur Rahman Sarkar ◽  
Md Ashfaque Ahemmed Khan ◽  
Mohammad Nazmul Islam Bhuiyan
Keyword(s):  
Right Ventricular ◽  
Tricuspid Valve ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Military Hospital ◽  
Base Line ◽  
Intact Ventricular Septum ◽  
Cardiac Catheterization Laboratory

Pulmonary atresia (PA) with intact ventricular septum is a rare, heterogeneous congenital heart defect with varying degrees of right ventricular and tricuspid valve hypoplasia and wide spectrum clinical features. Initial treatment at presentation is to establish systemic to pulmonary shunt or ductus stenting. The treatment options i.e. biventricular, one and half ventricular or one ventricular repair are often dictated by the degree of development of the tricuspid valve and right ventricle. X, 11 years old teenage girl got admitted to Combined Military Hospital (CMH) Dhaka at 5 months of age with severe cyanosis, respiratory distress, acidosis and shock. After immediate stabilization and work up, she was diagnosed as pulmonary atresia with intact ventricular septum, flapped atrial septal defect (ASD) and a small patent ductus arteriosus (PDA). On urgent basis, stenting of PDA done with 3.5 X 11 mm coronary stent at cardiac catheterization laboratory. Her SPO2 stepped up to 90 % from base line SPO2 from 50% following procedure. At 15 months of age she underwent intracardiac repair with short trans annular patch and ASD left open as a vent for RV. On follow up her ASD was getting larger and became desaturated and symptomatic. She was followed up for the right ventricular (RV) and tricuspid valve growth and ASD shunt. As her RV was developing nicely during follow up and achieved TV z score –1, ASD device closure was done with 18 mm Amplatzer ASD device at 5 years of age. She is now asymptomatic and thriving well. Cardiovasc. j. 2020; 13(1): 81-85

Pulmonary atresia with intact ventricular septum: management of, and outcomes for, a cohort of 210 consecutive patients

2004 ◽  
Vol 14 (3) ◽  
pp. 299-308 ◽  
Author(s):  
Umesh Dyamenahalli ◽  
Brian W. McCrindle ◽  
Cathy McDonald ◽  
Kalyani R. Trivedi ◽  
Jeffrey F. Smallhorn ◽  
...  
Keyword(s):  
Final Stage ◽  
Pulmonary Atresia ◽  
Left Ventricular ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Biventricular Repair ◽  
Initial Procedure ◽  
Pathology Reports ◽  
Over Time

Objectives:We sought to determine trends, and outcomes, for a cohort of patients with pulmonary atresia with intact ventricular septum born between 1965 and 1998.Background:Pulmonary atresia with intact ventricular septum is a complex lesion that remains a therapeutic challenge, particularly regarding the suitability for biventricular repair.Methods:We identified 210 consecutive patients, and reviewed their medical records, initial angiograms, and echocardiograms, along with the relevant surgical and pathology reports.Results:The mean initial Z-score for the diameter of the tricuspid valve was −0.99 ± 1.95, with Ebstein's malformation in 8%. A right ventricular dependent coronary arterial circulation was found in 23%. The proportion of patients who received treatment increased over time, although placement of an arterial shunt was the predominant initial procedure throughout the experience. At the last follow-up, 107 patients had not reached the planned final stage of their repair, and 79% of these had died. Of the 103 reaching the final stage of planned repair, 58 had undergone attempted biventricular repair, with 34% dying; 14 had undergone attempted one and a half ventricular repair, with 7% dying, and 31 had undergone attempted functionally univentricular repair, with 10% dying. Overall, survival was 57% at the age of 1 year, 48% at 5 years, and 43% at 10 years. Survival improved over time, with survival of 75% at 1 year, and 67% at 5 years, for patients born between 1992 and 1998. An earlier date of birth, the presence of Ebstein's malformation, and prematurity were all significant independent factors associated with decreased survival. A greater severity of coronary arterial abnormalities was significantly associated with a greater likelihood of left ventricular dysfunction during follow-up.Conclusions:The outcomes for patients born with pulmonary atresia with intact ventricular septum have improved over time, albeit that careful initial management, and better selection, is still indicated for those planned to undergo biventricular repair.

Myocardial infarct late after Fontan-type surgery in pulmonary atresia and intact ventricular septum: a must-know complication! About a case report

2021 ◽  
pp. 1-3
Author(s):  
Marie Wilkin ◽  
Jean Michel Rauzier ◽  
Caroline Ovaert
Keyword(s):  
Case Report ◽  
Right Ventricle ◽  
Myocardial Infarct ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Initial Assessment ◽  
Intact Ventricular Septum ◽  
Coronary Anatomy ◽  
Coronary Abnormalities

Abstract Coronary abnormalities are frequent in pulmonary atresia and intact ventricular septum, mainly in patients with a very diminutive right ventricle. They severely impact on early and late prognosis. We describe an 8-year-old girl who presented with myocardial ischaemia, late after uneventful Fontan completion. The importance of precise delineation of the coronary anatomy upon initial assessment and during follow-up is emphasised.

Left ventricular function in pulmonary atresia with intact ventricular septum after systemic-to-pulmonary arterial shunt

1994 ◽  
Vol 4 (2) ◽  
pp. 110-116
Author(s):  
Giuseppe Pacileo ◽  
Carlo Pisacane ◽  
Maria Giovanna Russo ◽  
Raffaele Calabrò
Keyword(s):  
Ejection Fraction ◽  
Surface Area ◽  
Body Surface Area ◽  
Body Surface ◽  
Pulmonary Atresia ◽  
Shape Index ◽  
Left Ventricular ◽  
Ventricular Septum ◽  
Intact Ventricular Septum

AbstractTo investigate the left ventricular systolic and diastolic function in patients with pulmonary atresia with intact ventricular septum without coronary-cardiac fistulas after a modified Blalock-Taussig shunt, 14 patients (age range 15 days-16.5 months, mean 4.03±5.6 months) and eight control subjects, matched for age, body surface area and heart rate were evaluated by cross-sectional and Doppler echocardiography. The follow-up interval after palliative procedures ranged from 12 days to 16.3 months (mean 3.67±5.6 months). Compared to controls, in the group of patients the ejection fraction was decreased (61±7% vs 68±5%, p=0.022) while the left ventricular end-diastolic volume indexed for body surface area was increased (72.7±10.8 cc/m246.1±12 cc/m2p=0.0001) with normal values of left ventricular mass indexed for body surface area (67.88±20.9 g/m2vs 76±10 g/m2p=NS). Mass-to-volume ratio was lower in patients with pulmonary atresia (0.95±0.38 vs 1.24±0.3, p=0.031). The left ventricular shape index was increased in all patients with pulmonary atresia (1.27±0.26 vs 1±0.01, p=0.009). A significant inverse correlation was noted between the ejection fraction and follow-up (r=−0.71, p=0.04). as well as between the ejection fraction and shape index (r=−0.76, p=0.048). Moreover, the patients with pulmonary atresia had decreased E/A velocity ratio (0.65±0.16 vs 1.35±0.90, p=0.009), decreased normalized peak filling rate (4.16±0.13 sv/s vs 6.88±0.68 sv/s, p=0.0001), increased peak A velocity (0.95±0.17 m/s vs 0.51±0.16 m/s, p=0.0001) and prolonged isovolumic relaxation time (46±5.4 ms vs 34±6.2 ms, p=0.0001) and deceleration time (196.4±32.2 ms vs 116±21.4 ms, p=0.0001). There was a good correlation between the normalized peak filling rate and follow-up (r=−0.80, p=0.04). These data show a progressive compromise of the left ventricular systolic and diastolic function in patients with pulmonary atresia with intact ventricular septum without ventriculocoronary fistulas who had undergone systemic-to-pulmonary arterial shunting. Thus, an earlier biventricular or Fontan type procedure should be recommended.

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