scholarly journals Prevalence and Spectrum of Complex Congenital Heart Disease in the Neonatal Intensive Care Unit at high Altitude in China

2020 ◽  
Author(s):  
Jing-Jing Li ◽  
Xiao-Rong Wang ◽  
Yuan Liu ◽  
Guo-Dong Zhao ◽  
Ting Dai ◽  
...  

Abstract Background: Previous studies from high altitudes have reported significantly higher prevalence of congenital heart disease (CHD), consisting almost solely of simple CHD. Little is known about the occurrence of complex CHD. Neonates with complex CHD are likely admitted to NICU. We examined the prevalence and spectrum of complex CHD in NICU in order to depict a truer picture of CHD at high altitude.Methods: We reviewed charts of 4,214 neonates admitted to NICU in Qinghai province (average altitude 3,000m). Echocardiography was performed in 1,943 babies when CHD was suspected based on clinical examinations.Results: CHD was diagnosed in 1,093(56.3% of echoed babies). Mild CHD in 96.8%(1058 babies). Moderate CHD in 0.8%(9) included 1(0.1%) large secundum atrial septal defect, 3(0.3%) moderate pulmonary stenosis, 2(0.2%) aortic stenosis and 3(0.3%) partial anomalous pulmonary venous connection. Severe CHD in 2.4%(26) included 6(0.5%) complete atrioventricular septal defect, 5(0.5%) complete transposition of the great arteries, 5(0.5%) hypoplastic right heart, 3(0.3%) hypoplastic left heart, 3(0.3%) double outlet right ventricle, 3(0.3%) tetralogy of Fallot, 2(0.2%) truncus arteriosus, 2(0.2%) total anomalous pulmonary venous connection, 2(0.2%) severe aortic stenosis, 2(0.2%) Interrupted aortic arch and 2(0.2%) severe pulmonary stenosis and 1(0.1%) single-ventricle abnormality. At two-years follow-up in 737(67.4%) patients, 18(90%) with severe CHD and 38(5.3%) with mild and moderate CHD died, and 15 underwent cardiac surgery with 1 early death.Conclusions: At high altitude, a wide spectrum of CHD exists, with many heretofore unreported complex CHD. There is urgent need for routine echocardiography and early interventions in newborns particularly in NICU.

2001 ◽  
Vol 41 (5) ◽  
pp. 237
Author(s):  
Teddy Ontoseno

There were 40 adult congenital heart disease (CHD) patients seen in the Cardiology Division during 1 year (February 1993 - February 1994). The most frequently seen defect was atrial septal defect; however there were also cases with patent ductus arteriosus, pulmonary stenosis, ventricular septal defect, and tetralogy of Fallot. Hemodynamic disorder, serious hindrance to education achievement, and occupational threat due to limited physical capabilities as well as malnutrition are some of prominent issues to be closely anticipated. In general the older the patients the more serious hemodynamic disorder they suffer due CHD. It is worth thinking how to improve the quality of life of CHD patients who succeed to live their adult lives and minimize any possible fatal complication risks.


2019 ◽  
Vol 3 (4) ◽  
pp. 1-4
Author(s):  
Julia Illner ◽  
Holger Reinecke ◽  
Helmut Baumgartner ◽  
Gerrit Kaleschke

Abstract Background Adults with complex congenital heart disease palliated with systemic-to-pulmonary artery shunts have become rare and represent a particularly challenging patient group for the cardiologist. One of the complications and causes of severe clinical deterioration during long-term follow-up are progressive obstruction or total occlusion of the shunt. The risk for surgical intervention is frequently high and catheter intervention may be complicated by complex anatomy and shunt calcification. Case summary We report the case of a 47-year-old man with uncorrected (palliated) pulmonary atresia and ventricular septal defect who presented with progressive cyanosis (oxygen saturation 69%) and decreasing exercise capacity. Computed tomography revealed a totally occluded modified left Blalock–Taussig (BT) shunt and a severely stenosed central shunt (Waterston–Cooley) in a patient with confluent but hypoplastic pulmonary arteries and multiple major aortic pulmonary collaterals. Due to a high operative risk, an interventional, percutaneous approach was preferred to re-do surgery. From a radial access the calcified BT shunt could be crossed with a hydrophilic guidewire. Then, a rotational thrombectomy, balloon dilatation, and bare-metal stenting at the proximal and distal anastomoses were performed. Post-interventionally, peripheral oxygen saturation increased from 69% to 82%. Clopidogrel was administered for 1 month after bare-metal stenting. At 1-year follow-up, the BT shunt was still patent on echocardiography and exercise tolerance markedly improved. Discussion This case highlights the benefit of percutaneous rotational thrombectomy followed by stenting of chronically occluded systemic-to-pulmonary artery shunts for further palliation in adult patients with complex congenital heart disease not suitable for surgical repair.


2018 ◽  
Vol 26 (7) ◽  
pp. 756-759 ◽  
Author(s):  
Hua Chun ◽  
Yan Yue ◽  
Yibin Wang ◽  
Zhaxi Dawa ◽  
Pu Zhen ◽  
...  

Background Previous small sample studies suggested that elevated altitudes might be associated with the incidence of cardiovascular diseases. However, it remains uncertain whether high altitudes (over 3000 m above sea level) are related to congenital heart disease. We therefore explored the prevalence of congenital heart disease in a large cohort of students in the world's largest prefecture-level city with the highest altitude. Methods This cross-sectional study included 84,302 student participants (boys 52.12%, girls 47.88%, with an average age of 10.62 ± 3.33 years). Data were extracted from the screening results among different altitude area schools in Nagqu from June 2016 to August 2017. Students were first screened by performing a physical examination consisting of cardiac auscultations and clinical manifestation screenings. An echocardiography was performed to confirm and identify the subtype of congenital heart disease. Results The prevalence of congenital heart disease among students in Nagqu, Tibet, was 5.21‰ (439 cases). The most common congenital heart disease type was patent ductus arteriosus, representing 66.3% of congenital heart diseases diagnosed in this study, followed by atrial septal defect and ventricular septal defect, representing 20.3% and 9.1% of congenital heart diseases, respectively. Students living in higher altitudes were significantly more prone to have congenital heart disease than students in locations with lower altitudes. The prevalence of congenital heart disease in girls was found to be higher than that of boys. Conclusions The correlation between congenital heart disease and increased altitude is noteworthy. This study's results are the first big data epidemiological investigation to confirm that high altitude is a significant environmental risk factor for congenital heart disease, especially patent ductus arteriosus. Furthermore, the results provide additional support to make a diagnostic and treatment plan to prevent congenital heart disease in high altitude areas.


2019 ◽  
Vol 12 (10) ◽  
pp. e231261
Author(s):  
Jeyaraj Durai Pandian ◽  
Ivy Anne Sebastian ◽  
Ashlee Sidhu

A 65-year-old woman presented to the emergency department with an acute onset of left-sided hemiparesis and slurred speech for 6 hours. Physical examination was notable for clubbing and peripheral cyanosis. Brain MRI showed an acute infarct involving the right gangliocapsular region. Investigations showed an elevated haemoglobin level, a haematocrit level of >65% and thrombocytopaenia. Transthoracic echocardiography revealed tricuspid atresia, ventricular septal defect and atrial septal defect. A diagnosis of cyanotic congenital heart disease was made. Studies have indicated an association between raised haematocrit and vascular occlusive episodes in patients with polycythaemia vera. However, a relationship between raised haematocrit due to polycythaemia secondary to complex congenital heart disease and vascular thrombosis has not been reported. The improvement of neurological deficits in our patient coincided with a decline in haematocrit. The objective of this report was to highlight an association between elevated haematocrit and ischaemic stroke in patients with secondary polycythaemia due to complex congenital heart disease.


2016 ◽  
Vol 44 (5) ◽  
pp. 206
Author(s):  
Wanty Sahli ◽  
J M Ch Pelupessy

Tetralogy of Fallot (TF) classically consistsof the combination of right ventricularoutflow obstruction (pulmonary stenosis),ventricular septal defect (VSD), overridingaorta, and right ventricular hypertrophy. Thedegree of pulmonary stenosis and VSD determine thevariety of clinical manifestations.This type of congenital heart disease accountsfor about 10% of all congenital cardiac deformitiesand is the most common cyanotic lesion after thefirst year of life. Cerebral abscess is a serious com-plication in TF and is usually seen after the age of 2years.


PEDIATRICS ◽  
1964 ◽  
Vol 33 (4) ◽  
pp. 562-570
Author(s):  
Leonard C. Harris ◽  
Mary Ellen Haggard ◽  
Luther B. Travis

Sickle cell disease, characterized by protean manifestations, has been confused frequently with rheumatic fever and congenital heart disease. Though it has been described in combination with rheumatic mitral stenosis, little on no consideration has been given to the association of sickle cell disease with congenital heart disease. This communication describes the occurrence of hemoglobin S disease in combination with congenital heart disease in three patients, the lesions being an atrial septal defect of the secundum type in one and pulmonary stenosis in two other patients. The congenital cardiac abnormalities were repaired under cardiopulmonary by-pass in the case of atrio-septal defect and the more severe case of pulmonary stenosis. Preparation for surgery consisted in the suppression of hemoglobin S formation by blood transfusions. During cardiopulmonary by-pass, further dilution of the hemoglobin S cells occurred so that their concentration in the patients' blood was negligible. Following surgery, it was necessary to administer greater amounts of intravenous fluid than usual to allow for the reduced ability to concentrate urine. Convalescence was unremarkable in each case.


Perfusion ◽  
2000 ◽  
Vol 15 (2) ◽  
pp. 161-168 ◽  
Author(s):  
Alfred H Stammers ◽  
Eric D Rauch ◽  
Lynne D Willett ◽  
Jamie W Newberry ◽  
Kim F Duncan

Severe coagulation defects often develop in neonates undergoing cardiac surgery, both as a result of the surgical intervention, and as pre-existing defects in the hemostatic mechanisms. The following case report describes a newborn patient with complex congenital heart disease and respiratory failure whose pre-operative coagulopathy was aggressively managed prior to surgical correction. A 5-day-old, 2.5 kg child presented with interrupted aortic arch, ventricular septal defect, atrial septal defect, and patent ductus arteriosus. On admission, he was in respiratory arrest suffering from profound acidemia. In addition, the child was hypothermic (30.1°C), septic ( Streptococcus viridans), and coagulopathic (disseminated intravascular coagulation - DIC). The patient was immediately intubated and initial coagulation assessment revealed the following: prothrombin time (PT) 48.9 s (international normalized ratio (INR) 15.7), activated partial thromboplastin time (aPTT) •106 s, platelet count 30 000 mm3, fibrinogen 15 mg dL-1 and antithrombin III (AT-III) 10%. Before cardiac surgery could be performed, the patient’s DIC was corrected with the administration of cryoprecipitate (15 ml), fresh frozen plasma (300 ml), and platelets (195 ml). In spite of the large transfusion of fresh frozen plasma, the AT-III activity, measured as a percentage, remained depressed at 33. Initial thromboelastographic (TEG) determination revealed an index of +2.02, and following 100 IU administration of an AT-III concentrate, declined to -2.32. Sequential TEG profiles were performed over several days, with the results used to guide both transfusion and medical therapy. The congenital heart defect correction was subsequently performed with satisfactory initial results, but the patient developed a fungal infection and expired on the 16th post-operative day. The present case describes techniques of coagulation management for a newborn with both a severe hemostatic defect and congenital heart disease.


2021 ◽  
Vol 8 ◽  
Author(s):  
Ming-Hui Zou ◽  
Fan Cao ◽  
Li Ma ◽  
Wei-Dan Chen ◽  
Wen-Lei Li ◽  
...  

Objectives: The management of atrial isomerism with complex congenital heart disease remains challenging. Experience has been largely obtained in advanced countries. The clinical diversity is greater in China. We evaluated the early- and medium-term outcomes of surgical treatment of these patients.Methods: We reviewed 86 patients of atrial isomerism with complex congenital heart disease undergoing varied surgeries in our center in 2008–2020. Cox regression models were used to analyze the risk factors for mortality.Results: There were 75 cases of right and 11 of left atrial isomerism. Eighty-three (96.5%) patients underwent single-ventricle staged palliation approach, with 10 early and 7 late deaths. The overall 1-, 5-, and 10-year survival rates were 84.7, 79.3, and 79.3%, respectively. Thirty-six (43.4%) patients completed the Fontan procedure with median age of 48 months and freedom from death or Fontan failure at 1-, 5-, and 8-years were 94.4, 87.4, and 80.7%, respectively. Concomitant total anomalous pulmonary venous connection [hazard ratio (HR): 5.15 (1.95–12.94), p = 0.008], more than moderate atrioventricular valve regurgitation [HR: 4.82 (2.42–6.79), p = 0.003], and the need for first-stage palliative surgery [HR: 4.58 (1.64–10.76), p = 0.015] were independent risk factors for mortality.Conclusions: Despite even greater clinical diversity, the surgical outcomes of atrial isomerism with complex congenital heart disease are improving in China. The early and intermediate outcomes are comparable to many previous reports. Concomitant total anomalous pulmonary venous connection, moderate or severe atrioventricular valve regurgitation, and the need for a first-stage palliative surgery are still independent risk factors for mortality.


Author(s):  
Mohamad El‐Chouli ◽  
Grímur Høgnason Mohr ◽  
Casper N. Bang ◽  
Morten Malmborg ◽  
Ole Ahlehoff ◽  
...  

Background We describe calendar time trends of patients with simple congenital heart disease. Methods and Results Using the nationwide Danish registries, we identified individuals diagnosed with isolated ventricular septal defect, atrial septal defect, patent ductus arteriosus, or pulmonary stenosis during 1977 to 2015, who were alive at 5 years of age. We reported incidence per 1 000 000 person‐years with 95% CIs, 1‐year invasive cardiac procedure probability and age at time of diagnosis stratified by diagnosis age (children ≤18 years, adults >18 years), and 1‐year all‐cause mortality stratified by diagnosis age groups (5–30, 30–60, 60+ years). We identified 15 900 individuals with simple congenital heart disease (ventricular septal defect, 35.2%; atrial septal defect, 35.0%; patent ductus arteriosus, 25.2%; pulmonary stenosis, 4.6%), of which 75.7% were children. From 1977 to 1986 and 2007 to 2015, the incidence rates increased for atrial septal defect in adults (8.8 [95% CI, 7.1–10.5] to 31.8 [95% CI, 29.2–34.5]) and in children (26.6 [95% CI, 20.9–32.3] to 150.8 [95% CI, 126.5–175.0]). An increase was only observed in children for ventricular septal defect (72.1 [95% CI, 60.3–83.9] to 115.4 [95% CI, 109.1–121.6]), patent ductus arteriosus (49.2 [95% CI, 39.8–58.5] to 102.2 [95% CI, 86.7–117.6]) and pulmonary stenosis (5.7 [95% CI, 3.0–8.3] to 21.5 [95% CI, 17.2–25.7]) while the incidence rates remained unchanged for adults. From 1977–1986 to 2007–2015, 1‐year mortality decreased for all age groups (>60 years, 30.1%–9.6%; 30–60 years, 9.5%–1.0%; 5–30 years, 1.9%–0.0%), and 1‐year procedure probability decreased for children (13.8%–6.6%) but increased for adults (13.3%–29.6%) were observed. Conclusions Increasing incidence and treatment and decreasing mortality among individuals with simple congenital heart disease point toward an aging and growing population. Broader screening methods for asymptomatic congenital heart disease are needed to initiate timely treatment and follow‐up.


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