CREATION OF A TEMPORARY ARTIFICIAL DUCTUS FOR THE SURGICAL CORRECTION OF VENTRICULAR SEPTAL DEFECTS ASSOCIATED WITH SEVERE PULMONARY HYPERTENSION

The article "Early Surgical Correction of Large Ventricular Septal Defects" by Sigmann, Stern, and Sloan which appeared in the January issue of Pediatrics (39:4, 1967) again illustrates the great concern in properly dealing with infants having such defects. Their 30% mortality in infants under 2 years of age with severe pulmonary hypertension is truly a remarkable surgical feat. However, as indicated by Dr. Nadas, in the preceding commentary, this must be weighed against the results of medical therapy and palliative surgical procedures.

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Pulmonary arterial changes in patients with ventricular septal defects and severe pulmonary hypertension

Pediatric Cardiology ◽

10.1007/bf02424988 ◽

1986 ◽

Vol 7 (3) ◽

pp. 147-154 ◽

Cited By ~ 27

Author(s):

Ruthellen Fried ◽

George Falkovsky ◽

Jane Newburger ◽

A. I. Gorchakova ◽

Marlene Rabinovitch ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Severe Pulmonary Hypertension ◽

Ventricular Septal Defects ◽

Septal Defects ◽

Pulmonary Arterial

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Abstract 15801: Early and Late Risk of Mortality or Development of Severe Pulmonary Hypertension Following Closure of Ventricular Septal Defects in Children With Severe Pulmonary Hypertension and Elevated Pulmonary Vascular Resistance Using Traditional or Uni-directional Flap Valve Patch Closure

Circulation ◽

10.1161/circ.142.suppl_3.15801 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

William M Novick ◽

Oleksandr Golovenko ◽

Vasyl Lazorhyshynets ◽

Vitaly Dedovich ◽

Iryna Perepeka ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Vascular Resistance ◽

Vascular Resistance ◽

Severe Pulmonary Hypertension ◽

Ventricular Septal Defects ◽

Risk Of Death ◽

Septal Defects ◽

Number Of Patients ◽

Flap Valve

Introduction: Children with large ventricular septal defects, severe pulmonary hypertension (PHT), and elevated pulmonary vascular resistance (PVR) are frequently denied operation in low and middle-income countries secondary to concerns of high mortality and development of severe pulmonary hypertension following operation, early and late. Hypothesis: We hypothesized that closure with a uni-directional flap valve patch (DP) would provide lower mortality and better survival without severe PHT. Methods: From 5/96 - 12/2016 all patients undergoing VSD closure with severe PHT and elevated PVR by either traditional (TP) or DP closure were followed annually. Baseline and O 2 provocation catheterization were performed before operation. Echocardiograms were performed at discharge and follow-up. The decision on TP or DP closure was based upon age and surgeon preference. Beginning in 2005 all patients regardless of technique received sildenafil 3 mgs/kg/day for 3 months before operation. Results: The number of patients receiving operation was 129, of which 40 received the DP. Males were 66/129, age and weight differed significantly for DP group compared to the TP group (7.5 vs 2.6 years, p<0.001 and 20 vs 11.5 kgs, p<0.001).Catheterization baseline data, PVR 9.0 vs 7.4 for DP and TP respectively (p<0.001), negative vasoactive test 32.5% vs 13.5% for DP and TP respectively (p=0.016). Mortality was 1/40 for DP and 9/89 for TP. Pulmonary to Systemic systolic pressure ratio was lower at discharge vs baseline cath (0.38 vs 0.99, p<0.001). Predictors of late severe pulmonary hypertension were PVR/SVR >0.5, p<0.024; and a negative vasoactive test of <10% decrease, p<0.001. PHT at last follow-up, none or mild 86/115 (74.8%), moderate 16/115 (13.9%) or severe 13/115 (11.3%). Conclusions: DP patients had less risk of death and both groups had few patients with severe PHT at late follow-up..

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EARLY SURGICAL CORRECTION OF LARGE VENTRICULAR SEPTAL DEFECTS

PEDIATRICS ◽

10.1542/peds.39.1.4 ◽

1967 ◽

Vol 39 (1) ◽

pp. 4-13

Author(s):

Joan M. Sigmann ◽

Aaron M. Stern ◽

Herbert E. Sloan

Keyword(s):

Pulmonary Hypertension ◽

Septal Defect ◽

Pulmonary Infections ◽

Severe Pulmonary Hypertension ◽

Ventricular Septal Defects ◽

Specific Therapy ◽

Surgical Closure ◽

Early Correction ◽

Septal Defects ◽

Pulmonary Vascular Obstructive Disease

A significant number of babies born with large ventricular septal defects present early in life as problems in management of congestive heart failure, severe pulmonary hypertension, marked growth retardation, and recurrent pulmonary infections. Review of patients born since December 1954 with ventricular septal defects and followed at our institution allowed for comparison between infants treated surgically and those patients treated either medically or requiring no specific therapy. In 45 infants direct surgical closure of the ventricular defect was undertaken when supportive medical management alone appeared to be inadequate to insure survival or a normal life potential. There was an 80% survival with surgery. All 9 deaths occurred in patients having severe pulmonary hypertension; in some instances additional factors were believed to have contributed to the death of the infant. Nonetheless, surgical mortality appeared to be lower in the infants having severe pulmonary hypertension than among children with a comparable degree of hypertension who were operated on over the age of 2 years. Postmortem examination revealed pathologic changes of advanced pulmonary vascular obstructive disease at as early an age as 8 months. Direct surgical closure of the ventricular septal defect was chosen in preference to the pulmonary artery handing procedure because it not only offers an immediate means of helping the sick infant but also should obviate the necessity of a second major surgical procedure when the patient is older. It is further anticipated that early correction of the septal defect will prove effective in many instances in preventing the serious consequences of pulmonary hypertension.

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A Case of Osseocartilaginous Defects and Cardiovascular Anomalies: An Ambiguous Entity

Journal of Asian Pacific Society of Cardiology ◽

10.15420/japsc.2021.06 ◽

2022 ◽

Vol 1 ◽

Author(s):

Aritra Paul ◽

Aruni Ghose ◽

Debasish Banerjee ◽

Suvro Banerjee

Keyword(s):

Pulmonary Hypertension ◽

Short Stature ◽

Eisenmenger Syndrome ◽

Severe Pulmonary Hypertension ◽

Ventricular Septal Defects ◽

Cardiac Anomalies ◽

Septal Defects ◽

History Of ◽

Cardiovascular Anomalies

A 24-year-old man presented with a history of palpitation and haemoptysis. He had a short stature, cardiac anomalies and physical deformities, including polydactyly, clubbing, cataracts and cyanosis. Echocardiography was performed, revealing both atrial and ventricular septal defects, along with severe pulmonary hypertension and Eisenmenger syndrome.

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Identification and functional analysis of variations in ENG promoter from patients of isolated ventricular septal defects associated with pulmonary hypertension

The FASEB Journal ◽

10.1096/fasebj.2021.35.s1.02617 ◽

2021 ◽

Vol 35 (S1) ◽

Author(s):

Huan‐Xin Chen ◽

Qin Yang ◽

Si‐Qiang Zheng ◽

Guo‐Wei He

Keyword(s):

Functional Analysis ◽

Pulmonary Hypertension ◽

Ventricular Septal Defects ◽

Septal Defects

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Clinical course in children and adolescents with ventricular septal defect

Asian Cardiovascular and Thoracic Annals ◽

10.1177/0218492319867001 ◽

2019 ◽

Vol 27 (7) ◽

pp. 529-534

Author(s):

Noor Mohammad Noori ◽

Alireza Teimouri

Keyword(s):

Pulmonary Hypertension ◽

Ventricular Septal Defect ◽

Septal Defect ◽

Clinical Course ◽

Defect Size ◽

Eisenmenger Syndrome ◽

Ventricular Septal Defects ◽

Laboratory Findings ◽

Septal Defects

Background Ventricular septal defect, the most common congenital heart defect, is characterized by an opening between the ventricles. This study aimed to evaluate the clinical course and associations between the characteristics of ventricular septal defect. Methods This cross-sectional study was conducted on 1498 children with ventricular septal defects, aged <19 years, who were referred to our center between 2003 and 2018. The diagnosis was suspected from a combination of clinical and laboratory findings, and confirmed by transthoracic echocardiography. Results Of the 1498 children, 54.9% were boys, 78.4% of defects were perimembranous, 30.4% of patients had pulmonary hypertension, 67.5% had regular follow-up, and 76 (5.1%) had complications including 28 (1.9%) with Eisenmenger syndrome; 10 died due to Eisenmenger syndrome during follow-up. The defects closed spontaneously in 38.9% and after surgery in 20.9%. Boys tended to have more perimembranous, inlet, and outlet forms (56.40%, 50.60%, 51.90%, respectively), whereas girls had more muscular types (51.80%). Most patients with pulmonary hypertension had perimembranous defects (83.10%). The majority of patients with pulmonary hypertension had large (63.40%) or moderate (36.60%) defects. Pulmonary hypertension had a significant association with defect size ( p < 0.001). After surgery, residual defects were found mostly in cases of large defects (84.60%). Most patients with spontaneous closure were younger than 4 years. Conclusion Almost four-fifths of children with ventricular septal defects had perimembranous types, and almost one-third had pulmonary hypertension which was associated with defect size. Two-fifths of the defects closed spontaneously. There was a low incidence of complications after surgery.

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