scholarly journals Pretriscuspid shunt and Eisenmenger syndrome: a deadly combination

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J Playan Escribano ◽  
T Segura De La Cal ◽  
L Dos Subira ◽  
M Lazaro Salvador ◽  
J.A Barbera Mir ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Statistical Significance ◽  
Functional Class ◽  
Clinical Group ◽  
Funding Source ◽  
Eisenmenger Syndrome ◽  
Confounding Variables ◽  
The Impact

Abstract Introduction Pulmonary arterial hypertension (PAH) is a common comorbidity in congenital heart disease (CHD) and significantly affects prognosis. Our aim was to investigate how the location of the shunt influences the outcome of these patients. Methods The REHAP registry is a Spanish multicentre voluntary registry of patients over 14 years of age, which includes patients with congenital heart disease and PAH, starting in 2007. Baseline characteristics, functional class, right catheterization data, treatment and survival were compared. We performed a cox regression model for assessing the impact of the type of shunt on prognosis, after looking for confusing and effect modifying variables. Results 664 patients were analyzed, their characteristics are detailed in the table. Mean follow up time was 10 years. We performed a survival analysis adjusted for the confounding variables: age (Hazard ratio (HR) 1.02 p<0.001), functional class (HR 2.45 p 0.001) and clinical group. The clinical group was also an effect-modifying variable, which means there is no unique effect (HR) of the type of shunt, instead it depends on the clinical group. We did a multiple regression analysis, adjusting for the confounding variables, to calculate the HR for each clinical group: in patients with Eisenmenger syndrome, the HR is 0.27 (p<0.001) for simple posttricuspid shunt and HR 0.38 (p<0.001) for complex posttricuspid shunt (reference group: pretricuspid shunt). In the others clinical groups, the type of shunt did not reach statistical significance. Conclusion The type of shunt significantly affects prognosis in Eisenmenger patients, with a much worse outcome for those with pretricuspid shunts. Functional class and clinical group are also closely related to outcome. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): MSD, GSK

Pulmonary hypertension and congenital heart disease: medical treatment and risk factors for survival

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J Playan Escribano ◽  
T Segura De La Cal ◽  
J Segovia Cubero ◽  
J Rueda Soriano ◽  
F.J Garcia Hernandez ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Cox Regression ◽  
Reference Group ◽  
Functional Class ◽  
Funding Source ◽  
Term Survival ◽  
First Line Therapy ◽  
Baseline Characteristics

Abstract Background Pulmonary arterial hypertension (PAH) is a common comorbidity in congenital heart disease (CHD) and significantly affects prognosis. There are four large clinical groups of CHD-related PAH: Eisenmenger syndrome, PAH associated with non-restrictive shunt, PAH associated with restrictive shunt and postoperative PAH. Our purpose was to study the clinical and prognostic differences among them. Methods The REHAP is a Spanish multicentre voluntary registry of patients over 14 years of age, which includes patients with CHD and PAH, starting in 2007. 664 patients were analyzed: Baseline characteristics, functional class, right catheterization data, treatment and survival were compared. Results 664 patients were analyzed: characteristics are detailed in the table. Patients with Eisenmenger were more frequently treated with oral monotherapy as a first line therapy and received less frequently prostanoids during the follow-up. Patients with Eisenmenger had significantly better prognosis, with the best long-term survival of the 4 groups. In a cox regression model, postoperative PAH has a 1.7 hazard ratio (HR) (reference group: Eisenmenger) after adjustment for age (HR 1.02 p 0,001), functional class (HR NYHA III-IV 2.3 p<0,001), sex (p 0.8) and pulmonary vascular resistance (p 0.7). Conclusion The clinical classification of PAH associated with CHD defines both the baseline characteristics and the prognosis of patients. Outcome relates closely to functional class and type of PAH–CHD. Eisenmenger group, which has the most severe hemodynamics, is the one with the best prognosis despite a less aggressive treatment. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Ferrer, Janssen

The impact of medical interventions on admission characteristics in children with congenital heart disease and cardiomyopathy

2020 ◽  
pp. 1-8
Author(s):  
Rohit S. Loomba ◽  
Jacqueline Rausa ◽  
Vincent Dorsey ◽  
Ronald A. Bronicki ◽  
Enrique G. Villarreal ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Length Of Stay ◽  
Congenital Heart ◽  
Unit Length ◽  
Channel Blockers ◽  
Regression Analyses ◽  
Converting Enzyme Inhibitors ◽  
Medical Interventions ◽  
The Impact

Abstract Introduction: Children with congenital heart disease and cardiomyopathy are a unique patient population. Different therapies continue to be introduced with large practice variability and questionable outcomes. The purpose of this study is to determine the impact of various medications on intensive care unit length of stay, total length of stay, billed charges, and mortality for admissions with congenital heart disease and cardiomyopathy. Materials and methods: We identified admissions of paediatric patients with cardiomyopathy using the Pediatric Health Information System database. The admissions were then separated into two groups: those with and without inpatient mortality. Univariate analyses were conducted between the groups and the significant variables were entered as independent variables into the regression analyses. Results: A total of 10,376 admissions were included these analyses. Of these, 904 (8.7%) experienced mortality. Comparing patients who experienced mortality with those who did not, there was increased rate of acute kidney injury with an odds ratio (OR) of 5.0 [95% confidence interval (CI) 4.3 to 5.8, p < 0.01], cardiac arrest with an OR 7.5 (95% CI 6.3 to 9.0, p < 0.01), and heart transplant with an OR 0.3 (95% CI 0.2 to 0.4, p < 0.01). The medical interventions with benefit for all endpoints after multivariate regression analyses in this cohort are methylprednisolone, captopril, enalapril, furosemide, and amlodipine. Conclusions: Diuretics, steroids, angiotensin-converting enzyme inhibitors, calcium channel blockers, and beta blockers all appear to offer beneficial effects in paediatric cardiomyopathy admission outcomes. Specific agents within each group have varying effects.

scholarly journals Double Inlet Left Ventricle with Eisenmenger Syndrome in an Adult – A Case Report

2017 ◽  
Vol 5 (1) ◽  
pp. 53-56
Author(s):  
Rahul Regi Abraham ◽  
Rahul Regi Abraham
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Congenital Heart ◽  
Pulmonary Stenosis ◽  
Eisenmenger Syndrome ◽  
Tertiary Center ◽  
History Of ◽  
Double Inlet Left Ventricle

Background: Patient diagnosed with double inlet left ventricle (prevalent in 5 – 10 in 100,000 newborns) complicated with Eisenmenger syndrome had a median survival age of 14 years without corrective surgery. Congenital heart disease such as this is usually treated by multiple surgeries during early childhood. A surgically uncorrected case in adults is not of common occurrence. Further, generalized itching after coming in contact with water (aquagenic pruritis) presented an interesting conundrum to treat. Case: A 29-year-old patient in India presented at a primary health care center with a history of difficulty breathing and discoloration of extremities since birth. He also gave a history of itching which commonly occurred after taking bath, hemoptysis and history of turning blue in color after birth. Patient had received no treatment besides regular phlebotomies. On examination, there was grade IV clubbing and conjunctival congestion. Cardiovascular examination revealed an enlarged heart, heaving apex beat and a pan-systolic murmur. A provisional diagnosis of a congenital cyanotic heart disease was made. Investigations revealed hemoglobin of 16.8g/dl. X–ray and electrocardiogram showed hypertrophy of the ventricles. An echocardiogram showed double inlet left ventricle with L-malposed vessels but without pulmonary stenosis. A final diagnosis of congenital heart disease; double inlet left ventricle, L-malposed vessels without pulmonary stenosis, Eisenmenger Syndrome and absolute erythrocytosis was made. Patient was advised for further management with a cardiologist in a tertiary center but the patient did not follow up. Conclusion: Unlike in high-income countries where most congenital heart diseases are detected and dealt with at birth whereas low-and middle-income nations often have to deal with cases that present much later and should often be included in the differential diagnosis. Inability to follow up cases, centers that are poorly equipped and lack of facilities for investigations, patient’s lack of medical awareness, and financial restrictions are major barriers to providing optimal treatment.

scholarly journals Surgical treatment of congenital heart disease with Eisenmenger syndrome

2021 ◽  
Vol 18 (1) ◽  
pp. 14-18
Author(s):  
Rakhima Mekenbayeva ◽  
Ulugbek Mazhidov ◽  
Yerbol Aldabergenov
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Surgical Treatment ◽  
Congenital Heart ◽  
Eisenmenger Syndrome

scholarly journals Cyanotic congenital heart disease: Impact on growth and endocrine functions.

2021 ◽  
Vol 28 (10) ◽  
pp. 1477-1483
Author(s):  
Muhammad Sohail Arshad ◽  
Waqas Imran Khan ◽  
Arif Zulqarnain ◽  
Hafiz Muhammad Anwar-ul-Haq ◽  
Mudasser Adnan
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Pulmonary Stenosis ◽  
Tertiary Care ◽  
Cyanotic Congenital Heart Disease ◽  
P Value ◽  
Common Diagnosis ◽  
Significant Difference ◽  
The Impact

Objective: To find out the impact of Cyanotic Congenital Heart Disease (CCHD) on growth and endocrine functions at a tertiary care child healthcare facility of South Punjab. Study Design: Case Control study. Setting: Department of Pediatric Cardiology and Department of Pediatric Endocrinology, Institute of Child’s Health (ICH), Multan, Pakistan. Period: December 2018 to March 2020. Material & Methods: During the study period, a total of 53 cases of Echocardiography confirmed CCHD were registered. Along with 53 cases, 50 controls during the study period were also enrolled. Height, weight, body mass index (BMI) along with hormonal and biochemical laboratory investigations were done. Results: There was no significant difference between gender and age among cases and controls (p value>0.05). Most common diagnosis of CCHD among cases, 24 (45.3%) were Tetralogy of Fallot (TOF) followed by 9 (17.0%) transposition of the great arteries (TGA) with Ventricular Septal Defect (VSD) with Pulmonary Stenosis (PS). Mean weight of CCHD cases was significantly lower in comparison to controls (21.19+6.24 kg vs. 26.48+8.1 kg, p value=0.0003). Blood glucose was significantly lower among cases in comparison to controls (77.58+14.58 mg/dl vs. 87.25+11.82 mg/dl, p value=0.0004). No significant difference was found in between cases and controls in terms of various hormone levels studied (p value>0.05) except Insulin-like Growth Factor-1 (IGF-1) levels (p value<0.0001). Conclusion: Children with cyanotic congenital heart disease seem to have negative effects on nutrition and growth. Change in pituitary-adrenal axis is suspected while pituitary-thyroid axis seemed to be working fine among CCHD cases. Serum glucose and IGF-1 levels were significantly decreased among CCHD cases.

Sign in / Sign up

Export Citation Format

Share Document