Acute ischaemic stroke in secondary polycythaemia due to complex congenital cyanotic heart disease

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.

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Stenting of modified Blalock–Taussig shunt in adult with palliated pulmonary atresia and ventricular septal defect: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytz201 ◽

2019 ◽

Vol 3 (4) ◽

pp. 1-4

Author(s):

Julia Illner ◽

Holger Reinecke ◽

Helmut Baumgartner ◽

Gerrit Kaleschke

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Congenital Heart ◽

Septal Defect ◽

Pulmonary Atresia ◽

Complex Congenital Heart Disease ◽

Bare Metal

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.

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Pre-operative coagulopathy management of a neonate with complex congenital heart disease: a case study

Perfusion ◽

10.1177/026765910001500212 ◽

2000 ◽

Vol 15 (2) ◽

pp. 161-168 ◽

Cited By ~ 8

Author(s):

Alfred H Stammers ◽

Eric D Rauch ◽

Lynne D Willett ◽

Jamie W Newberry ◽

Kim F Duncan

Keyword(s):

Congenital Heart Disease ◽

Cardiac Surgery ◽

Heart Disease ◽

Congenital Heart ◽

Septal Defect ◽

Fresh Frozen Plasma ◽

Complex Congenital Heart Disease ◽

At Iii ◽

Fresh Frozen ◽

Frozen Plasma

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.

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

10.21203/rs.3.rs-62194/v1 ◽

2020 ◽

Author(s):

Jing-Jing Li ◽

Xiao-Rong Wang ◽

Yuan Liu ◽

Guo-Dong Zhao ◽

Ting Dai ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Aortic Stenosis ◽

High Altitude ◽

Congenital Heart ◽

Septal Defect ◽

Severe Aortic Stenosis ◽

Pulmonary Stenosis ◽

Complex Congenital Heart Disease ◽

Anomalous Pulmonary Venous Connection

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.

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Isolated Interrupted Aortic Arch- A Complex Congenital heart disease

Cardiovascular Journal ◽

10.3329/cardio.v4i1.9400 ◽

1970 ◽

Vol 4 (1) ◽

pp. 102-104

Author(s):

A Shariar ◽

MA Huq ◽

NU Zakia ◽

M Salim ◽

SA Islam

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Aortic Arch ◽

Congenital Heart ◽

Septal Defect ◽

Interrupted Aortic Arch ◽

Cardiac Anomaly ◽

Complex Congenital Heart Disease ◽

Type B ◽

Left Carotid Artery

Interrupted Aortic Arch (lAA) is the absence or discontinuation of a portion of the aortic arch. There are three types of Interrupted Aortic Arch,and they are classified according to the site of the interruption. lsolated IAA appears commonly in type B lAA,where the interruption occurs between the left carotid artery and the left subclavian artery.In such case there is no associated cardiac anomaly and the patient may not exhibit lethal form of presentation unless there is duct dependent circulation or ventricular septal defect. lsolated interrupted arch is relatively rare congenital anomaly and very few cases were reported in literature so far. Keywords: lnterrupted aortic arch; Complex congenital heart disease. DOI: http://dx.doi.org/10.3329/cardio.v4i1.9400 Cardiovasc. J. 2011; 4(1): 102-104

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Comorbidities with Congenital Heart Disease among Hospitalized Children in a Specialized Cardiac Hospital in Bangladesh

Cardiovascular Journal ◽

10.3329/cardio.v9i2.32418 ◽

2017 ◽

Vol 9 (2) ◽

pp. 83-89

Author(s):

Shahina Akhter Mita ◽

Md Salim ◽

Md Ataul Haque ◽

Abdullah Shahriar ◽

Md Nazneen Umme Zakia ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Septal Defect ◽

Complex Congenital Heart Disease ◽

Comorbid Condition ◽

Comorbid Conditions ◽

Transposition Of Great Arteries ◽

Different Types ◽

Valvular Lesion

Background: Congenital heart diseases (CHD) when associated with any comorbid condition may complicate the outcome of management. So to find out and treat comorbidities before definite treatment is essential for better outcome. Methods: This observational cross sectional study was conducted in the inpatient Department of Paediatric Cardiology at National Institute of Cardiovascular Diseases, Dhaka, during the period of January 2014 to January 2015 to identify the comorbid conditions of congenital heart disease.Results: A total of 794 children with different types of congenital heart disease were enrolled during the study period. Among them, 48.6% were simple CHD and 51.4% were complex CHD. Among simple CHD, ventricular septal defect (VSD) was commonest (27.2%), Tetralogy of Fallot (TOF) occupied the second position (20.5%). One child presented with Ectopia cardis. Among complex CHD most common anomalies were VSD with either atrial septal defect (ASD) or patent ductus arteriosus (PDA) or valvular lesion. Next common anomalies were TOF with PDA or ASD &/or pulmonary valvular abnormalities. Double outlet of right ventricle (DORV) with shunt and transposition of great arteries (TGA) with shunt also occur frequently. AV canal defect with or without valvular lesion presented in significant number. Among them 616 (77.6%) had different types of comorbid conditions. Among comorbidities, respiratory and acquired cardiac comorbidities were common (37.9% & 34.1% respectively). Other congenital or genetic comorbidities were in 11.2%. Multisystem involvement was in 9.8% cases.Conclusion: Pneumonia and heart failure were the most frequent comorbid condition among both simple and complex congenital heart disease. They were common among acyanotic heart disease. But cyanotic spell, acute stroke syndrome and brain abscess were common among cyanotic heart disease. Among genetic comorbidities Downs syndrome occupied the major part.Cardiovasc. j. 2017; 9(2): 83-89

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Abstract 4553: Pre-Operative Brain Injury in Newborn Infants with Transposition of the Great Arteries Occurs at Similar Rates to other Complex Congenital Heart Disease, and is not Related to Balloon Atrial Septostomy

Circulation ◽

10.1161/circ.118.suppl_18.s_909 ◽

2008 ◽

Vol 118 (suppl_18) ◽

Author(s):

Julia Gunn ◽

Lara Shekerdemian ◽

Rod Hunt ◽

Lee Coleman ◽

Ayton Hope ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Brain Injury ◽

Ischaemic Stroke ◽

Congenital Heart ◽

Pulmonary Atresia ◽

Complex Congenital Heart Disease ◽

Balloon Atrial Septostomy ◽

Increased Risk ◽

Atrial Septostomy

Introduction: Brain injury (BI) in the form of white matter injury (WMI) or ischaemic stroke, seen on magnetic resonance imaging (MRI), is common in neonates with congenital heart disease (CHD). It has previously been suggested that balloon atrial septostomy (BAS) may be an independent risk factor for pre-operative BI in neonates with transposition of the great arteries (TGA). Hypothesis : We hypothesised that: 1. The incidence of BI is similar in neonates with TGA and other severe CHD 2. The risk of pre-operative BI is not increased by BAS Methods: Neonates with TGA, hypoplastic left heart syndrome (HLHS) or pulmonary atresia (PA) were enrolled in a prospective study of BI related to CHD. Pre-operative brain MRI was performed in all patients. In those with TGA who underwent BAS, MRI occurred after septostomy. T1, T2 and diffusion-weighted images were obtained in axial and coronal planes. Scans were reviewed by blinded assessors, and were classified according to WMI (punctate lesions) or ischaemic stroke. Fisher’s Exact Test was used to compare rates of WMI and stroke in each patient group. Results: Sixty-six neonates (mean weight 3.4±0.5kg) were studied, of whom 44 had TGA, 13 had HLHS and 9 had PA. The median age at MRI was 7 days (range 1–31). Thirty-five of the neonates with TGA underwent BAS a median of 1 day (1–21) followed by MRI 7 days (2–12) later. BI was present in 17 (26%) patients. The table shows the incidence of BI in the sub-groups. Two neonates with a stroke also had WMI. Table showing MRI abnormalities in patient sub-groups. Neonates with TGA were not at increased risk of pre-operative BI compared with those with HLHS or PA (p=1.0). BAS did not increase the risk of pre-operative BI in neonates with TGA (p=1.0). Conclusions: Pre-operative BI occurs at a similar rate in neonates with TGA and other severe CHD. The risk of pre-operative BI in neonates with TGA is not increased by BAS.

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An Unusual Coronary Artery Complication after Surgery for a Complex Congenital Heart Disease: A Case Report

International Journal of Innovative Research in Medical Science ◽

10.23958/ijirms/vol06-i12/1302 ◽

2021 ◽

Vol 6 (12) ◽

pp. 919-923

Author(s):

Zaher Faisal Zaher, MD

Keyword(s):

Congenital Heart Disease ◽

Cardiac Surgery ◽

Heart Disease ◽

Coronary Artery ◽

Ventricular Septal Defect ◽

Congenital Heart ◽

Septal Defect ◽

Arterial Switch Operation ◽

Complex Congenital Heart Disease ◽

Switch Operation

Background: Unexpected events in cardiac surgery may increase morbidity and mortality, which may be more complicated and difficult to manage especially in redo complex congenital heart surgery in children. This report presents a case of unusual coronary artery complication after redo surgery for a complex congenital heart disease. Case Presentation: A female baby with ventricular septal defect, transposition of great arteries and coarctation of the aorta, underwent coarctation repair and pulmonary banding in neonatal period then at one year old, she underwent arterial switch operation and ventricular septal defect repair. In the pediatric cardiac intensive care unit, she required high doses of vasoactive inotropic drugs and echocardiography revealed sever right ventricular dysfunction without significant gradients or shunts. Accordingly, a diagnostic cardiac catheterization was performed and the aortic angiography revealed that the right coronary artery was not inadvertently transferred into the neo -aortic root during the step of arterial switch operation. The surgeon declared that he came upon confounding factors during the intraoperative assessment, which led him to identify a single coronary artery. Immediate reoperation performed to translocate the non-transferred right coronary artery into the neo aortic root and postoperatively the case required extracorporeal membrane oxygenation till she stabilized, and she was discharged in an acceptable condition. Conclusion: Early diagnosis and management of any complications related to coronary artery during pediatric cardiac surgery could lower risk and achieve favorable outcome.

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