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

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.

Clinical course in children and adolescents with ventricular septal defect

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.

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

1986 ◽  
Vol 7 (3) ◽  
pp. 147-154 ◽  
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

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 ◽  
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..

EARLY SURGICAL CORRECTION OF LARGE VENTRICULAR SEPTAL DEFECTS

PEDIATRICS ◽  
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.

Management of Infants with Ventricular Septal Defects

PEDIATRICS ◽  
1967 ◽  
Vol 40 (1) ◽  
pp. 144-145
Author(s):  
JOHN H. K. VOGEL ◽  
S. GILBERT BLOUNT
Keyword(s):  
Pulmonary Hypertension ◽  
Medical Therapy ◽  
Surgical Procedures ◽  
Surgical Correction ◽  
Severe Pulmonary Hypertension ◽  
Ventricular Septal Defects ◽  
Septal Defects ◽  
January Issue

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.

Etiology of Ventricular Septal Defects: An Epidemiologic Approach

PEDIATRICS ◽  
1985 ◽  
Vol 76 (5) ◽  
pp. 741-749
Author(s):  
Thomas B. Newman
Keyword(s):  
The United States ◽  
Incidence Rates ◽  
Identical Twins ◽  
Random Errors ◽  
Ventricular Septal Defects ◽  
Metabolic Disturbances ◽  
Cardiac Morphogenesis ◽  
Septal Defects ◽  
Wide Range ◽  
History Of

To investigate the recent 150% increase in the reported incidence of ventricular septal defects (VSDs) in the United States, the epidemiology of ventricular septal defects was examined. The apparent incidence of VSDs is highly dependent on case finding methods, and more complete diagnosis and reporting probably account for the increase in reported incidence. Variations in case ascertainment also account for the small differences in incidence in studies from different places. The several known risk factors for VSD, including a family history of congenital heart disease and exposure to certain drugs, infectious agents, and maternal metabolic disturbances, explain few cases. Incidence rates are similar in different races and seasons and are unrelated to maternal age, birth order, sex, and socioeconomic status. VSDs occur naturally in a wide range of mammals and in birds, which also have four-chambered hearts. Despite identical genes and similar prenatal environments, the concordance rate in identical twins is only about 10%. The consistency of incidence among individuals with widely differing genes and environments and the frequency of discordance in identical twins suggest that VSDs often occur as random errors in development, at a frequency largely determined by the complexity of normal cardiac morphogenesis. This hypothesis has two major implications: many VSDs are not preventable and parents need not feel responsible for VSDs in their children.

THE NATURAL HISTORY OF PULMONARY HYPERTENSION IN CHILDREN WITH VENTRICULAR SEPTAL DEFECTS ASSESSED BY SERIAL RIGHT-HEART CATHETERIZATION

PEDIATRICS ◽  
1961 ◽  
Vol 27 (4) ◽  
pp. 621-626
Author(s):  
Robert E. Stanton ◽  
Donald C. Fyler
Keyword(s):  
Natural History ◽  
Arterial Pressure ◽  
Pulmonary Arterial Pressure ◽  
Clinical Status ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Ventricular Septal Defects ◽  
Septal Defects ◽  
History Of ◽  
Pulmonary Arterial

The natural history of the pulmonary arterial pressure in 23 children with isolated ventricular septal defects has been evaluated by serial night heart catheterization. In none of the 23 patients was there a significant increase in pulmonary arterial pressure. Eight patients showed a significant decrease. The direction of shunts did not change between procedures, and the clinical status was stable. Our experience correlated well with the majority of institutions. It would appear that the natural course of pulmonary arterial pressure in ventricular septal defect is not commonly one of progressive rise.

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