scholarly journals Against all odds—late repair of multiple shunt lesions in a patient with Down syndrome: a case report

2021 ◽  
Vol 5 (7) ◽  
Author(s):  
Alexandra Arvanitaki ◽  
Katarzyna Januszewska ◽  
Edward Malec ◽  
Helmut Baumgartner ◽  
Hans-Gerd Kehl ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Septal Defect ◽  
Surgical Repair ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
Resistance Index ◽  
Late Presentation ◽  
Careful Consideration ◽  
Pulmonary Vascular Disease

Abstract Background Children with congenital heart defects (CHD) usually undergo elective surgical repair of haemodynamically relevant shunt lesions within the first year of life. Due to susceptibility for pulmonary arterial hypertension (PAH) in patients with Down syndrome, repair is usually aimed for no later than 6 months of life. However, with rising immigration from developing countries to Europe, more patients with unrepaired CHD are diagnosed at a later age. Anatomical repair may be precluded, when advanced pulmonary vascular disease has been established. Case summary We report a 39-month-old male patient with Down syndrome with a large non-restrictive perimembranous ventricular septal defect, a large patent ductus arteriosus, and a secundum-type atrial septal defect with a prominent left-to-right shunting. Haemodynamic assessment revealed only a mild increase of pulmonary artery pressures (mPAP) with low pulmonary vascular resistance index (PVRi). Vasodilator testing led to a further increase of the left-to-right shunt and decrease of PVRi, suggesting operability. After careful consideration, the patient underwent complete surgical repair with a good post-operative clinical outcome. Cardiac catheterization 6 months after corrective repair showed a normal mPAP. No signs of PAH have been detected in the medium-term follow-up. Discussion Expertise, increased physician awareness, and a thorough pre-operative multidisciplinary evaluation are paramount to determine the best treatment approach for patients, who may present late with multiple shunts, and—in our case—underlying Down syndrome. Long-term close post-surgical follow-up in an expert centre is warranted to promptly diagnose and treat a possible late presentation of PAH appropriately.

Congenital Cardiovascular Defects in Infants With Down Syndrome

1989 ◽  
Vol 11 (4) ◽  
pp. 99-100
Author(s):  
Edward B. Clark
Keyword(s):  
Down Syndrome ◽  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
Atrioventricular Canal ◽  
Cardiac Evaluation ◽  
Cardiovascular Defects ◽  
Complete Atrioventricular ◽  
Patent Ductus

Editor's note: In response to a reader's question about cardiac evaluation of patients with Down syndrome, we asked Dr Clark for a review and recommendation for workup by the general pediatrician. RJH Infants with Down syndrome are at 50% risk for congenital cardiovascular malformations1 compared with a 0.3% (3 of 1000) risk for infants with normal chromosomes.2 These data confirm the original estimates of Rowe and Uchida3; other estimates have ranged from 20% to 70%. The types of heart defects found in persons with Down syndrome are severe: complete atrioventricular canal, ventricular septal defect, partial atrioventricular canal, atrial septal defect, tetralogy of Fallot, and patent ductus arteriosus.4

scholarly journals Congenital Cardiovascular Anomalies among Cases of Down Syndrome: A Hospital Based Review of Cases in TikurAnbessa Specialized Hospital, Ethiopia

1970 ◽  
Vol 29 (2) ◽  
Author(s):  
Ahmed Muntha ◽  
Tamirat Moges
Keyword(s):  
Down Syndrome ◽  
Ventricular Septal Defect ◽  
Patent Ductus Arteriosus ◽  
Septal Defect ◽  
Ductus Arteriosus ◽  
Cardiac Defects ◽  
Cardiac Anomalies ◽  
Lost To Follow Up ◽  
Patent Ductus

BACKGROUND: In developing countries, infants with Down syndrome and cardiac defect are at increased risk of dying. Congenital heart diseases occur in 40-50% of affected infants.Endocardial cushion defect accounts for the most. Pattern of cardiac defects in Down syndrome vary with ethnicity. The current study aims to determine pattern of cardiac defects and survival of patients in our institution.METHODS: Hospital based review of cases, between April 2010 and may 2015 were made. Data were analyzed using SPSS version 20 software quantitatively with plotted Kaplan Meier survival curve done. RESULTS: Down syndrome cases, 53 male and 63 females with cardiac anomalies, were described. Patent ductus arteriosus occurs in 57(36.5%), Ventricular septal defect in 31(19.9%), Atrial septal defect in 30(19%), Atrio-ventricular septal defect in29(18.6%),Tetralogy of Fallot in 4(2.6%) and others in 5(3.2%) cases. Cases were alive, lost to follow-up and died in 59, 35 and 22 cases, respectively. The overall death rate was 19% and two-third of them died during infancy with females dying at an earlier age than males. Pulmonary hypertension, hypothyroidism and gastrointestinal disorders were diagnosed in 46, 21 and 4 of the cases, respectively. Reasons for lost to follow-up were discussed.CONCLUSION: The pattern of cardiac anomalies in our study among Down syndrome cases is dominated by Patent ductus arteriosus while, the overall survival of cases is guarded as majority of deaths occurred during infancy

scholarly journals Down syndrome and congenital heart disease: perioperative planning and management

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Dennis R. Delany ◽  
Stephanie S. Gaydos ◽  
Deborah A. Romeo ◽  
Heather T. Henderson ◽  
Kristi L. Fogg ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Heart Defects ◽  
Careful Consideration ◽  
Children With Down Syndrome ◽  
Perioperative Planning

AbstractApproximately 50% of newborns with Down syndrome have congenital heart disease. Non-cardiac comorbidities may also be present. Many of the principles and strategies of perioperative evaluation and management for patients with congenital heart disease apply to those with Down syndrome. Nevertheless, careful planning for cardiac surgery is required, evaluating for both cardiac and noncardiac disease, with careful consideration of the risk for pulmonary hypertension. In this manuscript, for children with Down syndrome and hemodynamically significant congenital heart disease, we will summarize the epidemiology of heart defects that warrant intervention. We will review perioperative planning for this unique population, including anesthetic considerations, common postoperative issues, nutritional strategies, and discharge planning. Special considerations for single ventricle palliation and heart transplantation evaluation will also be discussed. Overall, the risk of mortality with cardiac surgery in pediatric patients with Down syndrome is no more than the general population, except for those with functional single ventricle heart defects. Underlying comorbidities may contribute to postoperative complications and increased length of stay. A strong understanding of cardiac and non-cardiac considerations in children with Down syndrome will help clinicians optimize perioperative care and long-term outcomes.

scholarly journals Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service

2014 ◽  
Vol 32 (2) ◽  
pp. 159-163 ◽  
Author(s):  
Felipe Alves Mourato ◽  
Lúcia Roberta R. Villachan ◽  
Sandra da Silva Mattos
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Diseases ◽  
Heart Defects ◽  
Descriptive Analysis ◽  
Atrioventricular Septal Defect

OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.

Prevalence of Culture-Negative Fever in Infants With Down Syndrome Undergoing Cardiac Surgery

2019 ◽  
Vol 10 (5) ◽  
pp. 599-603
Author(s):  
Jeffrey Vergales ◽  
Michael D. Seckeler ◽  
Joshua Chew ◽  
James Gangemi
Keyword(s):  
Down Syndrome ◽  
Cardiac Surgery ◽  
Septal Defect ◽  
Surgical Repair ◽  
Area Under The Curve ◽  
Control Group ◽  
Chromosomal Anomalies ◽  
Retrospective Case ◽  
Postoperative Fever ◽  
Culture Negative

Background: Children with Down syndrome (DS) often have congenital heart disease that requires surgical repair in the first year. Anecdotally, we have noted that patients with DS seem to have a higher rate of culture-negative postoperative fever. The objective was to evaluate the prevalence of postoperative fever and recovery among patients with DS undergoing cardiac surgery. Methods: We conducted a retrospective, case–control study of all patients at our institution less than one year of age with DS undergoing surgical repair of an atrioventricular septal defect or ventricular septal defect between 2010 and 2016. The control group was patients with no chromosomal anomalies who were age and surgery matched to the DS group. Temperatures were recorded for the first 72 hours postoperatively, with duration and degree of fever being assessed using the area under the curve. Results: Patients with DS (n = 34) had a significantly higher prevalence of fever than the control group (59% vs 24%, P = .003), longer ventilator time, and longer length of stay. Among the DS group, those who developed fever tended to be older at the time of surgery (146 ± 63 vs 103 ± 45 days, P = .04). The DS group with fever had similar cardiopulmonary bypass times, intensive care unit and total lengths of stay, ventilator days, and hospital costs compared to patients with DS without fever. Conclusions: Patients with DS have a higher incidence of culture-negative fever within the first 72 hours. The presence of fever in these patients, however, does not affect their overall postoperative course.

scholarly journals Successful percutaneous occlusion of a large left circumflex coronary artery fistula draining into the coronary sinus using a ventricular septal defect occluder: a case report

2020 ◽  
Vol 4 (2) ◽  
pp. 1-6
Author(s):  
Anastasia D Egorova ◽  
Peter Ewert ◽  
Martin Hadamitzky ◽  
Andreas Eicken
Keyword(s):  
Coronary Artery ◽  
Ventricular Septal Defect ◽  
Coronary Sinus ◽  
Septal Defect ◽  
Heart Defects ◽  
Coronary Artery Fistula ◽  
Circumflex Coronary Artery ◽  
Left Circumflex Coronary Artery ◽  
Complex Anatomy

Abstract Background Coronary artery fistula (CAF) is a congenital anomaly of the coronaries that can lead to significant intracardiac shunting and myocardial ischaemia. Case summary We describe the case of a 15-year-old male with an incidentally documented precordial cardiac murmur. An evidently dilated coronary sinus (CS) on transthoracic echocardiography prompted further investigation. A computed tomography (CT) revealed the presence of a large CAF from the left circumflex coronary artery to the CS. No other structural heart defects were detected. A haemodynamically significant intracardiac shunt was confirmed during cardiac catheterization, and it was decided to close the fistula. This was successfully performed using a ventricular septal defect (VSD) occluder (Konar 10-8, Lifetech Scientific) that was deployed through a 6 Fr right coronary guiding catheter. A partial thrombotic occlusion of the CS behind the closure device was noted during follow-up which led to anticoagulation in a higher target INR range and concomitant start of low dose carbasalate calcium to reduce further retrograde thrombus extension. Patient is doing well at over 1 year of follow-up, and no further thrombotic extension into the CS was seen on a recent CT. Discussion This report illustrates the diagnostic workup and a percutaneous treatment strategy of a CAF using a VSD occluder. We also describe a not previously reported complication, thrombotic CS occlusion. Improving transcatheter techniques and marketing of novel devices with a broad spectrum of applications can offer new opportunities for treating CAF and avoiding surgical correction often involving cardiopulmonary bypass, reserving this option for patients with complex anatomy or failed transcatheter closure.

Optimal surgical approach for repair of aortopulmonary window

2001 ◽  
Vol 11 (4) ◽  
pp. 385-390 ◽  
Author(s):  
Chee-Chin Hew ◽  
Emile A. Bacha ◽  
David Zurakowski ◽  
Pedro J. del Nido ◽  
John E. Mayer ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Coronary Artery ◽  
Aortic Coarctation ◽  
Septal Defect ◽  
Surgical Repair ◽  
Patient Survival ◽  
Aortopulmonary Window ◽  
Single Institution ◽  
Hospital Deaths

Background: This is a review of the experience over 26 year in a single institution with surgical repair of aortopulmonary window. Methods: Between July 1973 and March 1999, 38 patients underwent surgery for aortopulmonary window at a median age of 5 weeks, and with a median weight of 3.9 kg. Median follow-up was 6.6 years, with a range from 0.8 to 26 years. Additional defects were present in 25 (65%) patients, including interruption of the aortic arch in 7, tetralogy of Fallot in 7, ventricular septal defect in 5, functionally univentricular anatomy in 3, aortic coarctation in 2, and anomalous origin of a coronary artery in 1. We approached via an aortotomy in 45%, an incision through the defect in 31%, and using a pulmonary arteriotomy in 24% of patients. Closure was achieved using a single patch in 30 patients (79%). Results: There were 3 (7.9%) in-hospital deaths. Actuarial patient survival was 88% at 10 years. Three patients required reinterventions for stenoses of the great arteries. Freedom from any reintervention was 70% at 10 years. By multivariate analysis, the approach through a pulmonary arteriotomy was shown to result in a higher need for reintervention (p = 0.01). Conclusions: Repair of aortopulmonary window can be done with excellent results. A pulmonary arteriotomy should be avoided.

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