scholarly journals Isolated Interrupted Aortic Arch- A Complex Congenital heart disease

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

scholarly journals Endograft rescue of compromised interposition aortic graft in an adult patient with congenital heart disease

2018 ◽  
Vol 2018 (1) ◽  
Author(s):  
Jesse W Lee ◽  
Kanishka Ratnayaka ◽  
Howaida G El-Said ◽  
John W Moore
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Adult Patient ◽  
Aortic Arch ◽  
Congenital Heart ◽  
Bare Metal Stents ◽  
Interrupted Aortic Arch ◽  
Complex Congenital Heart Disease ◽  
Metal Stents ◽  
Pseudoaneurysm Formation

In a 19-year-old male with interrupted aortic arch and complex congenital heart disease, we report percutaneous repair of a compromised aortic conduit. The patient had aortic arch repair in childhood utilizing a 12 mm Hemashield Dacron conduit. CT angiography showed multiple segments of this conduit were dilated to 16 mm suggesting conduit degeneration and failure with pseudoaneurysm formation. We utilized a self-expanding aortic endograft supported by internal placement of bare metal stents to repair the conduit. Our repair was guided by 3D rotational angiography. This adult patient with complex congenital heart disease and interrupted aortic arch is an example of patients in whom endograft repair of compromised aortic conduits presents a much lower risk alternative than surgical revision. 

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

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.

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

2019 ◽  
Vol 12 (10) ◽  
pp. e231261
Author(s):  
Jeyaraj Durai Pandian ◽  
Ivy Anne Sebastian ◽  
Ashlee Sidhu
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Ischaemic Stroke ◽  
Congenital Heart ◽  
Septal Defect ◽  
Brain Mri ◽  
Neurological Deficits ◽  
Complex Congenital Heart Disease ◽  
Haematocrit Level ◽  
Polycythaemia Vera

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.

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

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
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.

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 ◽  
...  
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.

Reconstruction of the aortic arch in an adolescent female with complex congenital heart disease

1994 ◽  
Vol 4 (2) ◽  
pp. 164-167
Author(s):  
Rajiv Verma ◽  
Delores Danilowicz ◽  
Stephen Colvin
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Aortic Arch ◽  
Congenital Heart ◽  
Complex Congenital Heart Disease ◽  
Adolescent Female ◽  
Ventricular Septal Defects ◽  
Postoperative Course ◽  
Septal Defects ◽  
Complete Repair

AbstractCoarctations of the aortic arch are rare, and when found, they are often associated with other complex congenital heart disease. An adolescent female with multiple coarctations of the aortic arch and ventricular septal defects underwent complete repair, entailing replacement of the aortic arch with a homograft and closure of the ventricular septal defects. The postoperative course was complicated by a tear in the homograft, possibly related to sepsis. After its resolution, her hemodynamic results remain excellent.

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

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 Down’s syndrome occupied the major part.Cardiovasc. j. 2017; 9(2): 83-89

Critical Congenital Heart Disease Screening in NICU: Need for Revision and Standardization

2017 ◽  
Vol 34 (14) ◽  
pp. 1470-1476 ◽  
Author(s):  
Billie Short ◽  
Veena Manja ◽  
Satyan Lakshminrusimha ◽  
Nithi Fernandes
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulse Oximetry ◽  
Aortic Arch ◽  
Neonatal Intensive Care ◽  
Congenital Heart ◽  
Interrupted Aortic Arch ◽  
Screening Methods ◽  
Aortic Arch Anomalies ◽  
Critical Congenital Heart Disease

AbstractScreening for critical congenital heart disease (CCHD) at 24 to 48 hours after birth or before discharge in newborn nurseries using pulse oximetry is effective and is mandated by most states. However, there is no established protocol for screening in a neonatal intensive care unit (NICU), a setting where neonates are continuously monitored by pulse oximetry, hypoxemia from noncardiac causes is common, and echocardiograms are frequently obtained. CCHDs with hypoxemia are suspected on admission and investigated with an echocardiogram before a formal screen in the NICU. The most common CCHD lesions missed in a NICU setting are secondary targets of the screen, such as aortic arch anomalies (coarctation or interrupted aortic arch). The sensitivity of the current pulse oximeter–based CCHD screen to diagnose aortic arch anomalies is low. Given that infants are monitored with continuous pulse oximetry and frequent examinations, novel revisions to the current screening methods are necessary to detect asymptomatic NICU infants with aortic arch anomalies before discharge. Exclusions (whom to screen), technique (how to screen), and timing (when to screen) for primary and secondary targets of CCHD in the NICU are not known and require further research.

Right-Sided Aortic Arch

2010 ◽  
pp. 263-271
Author(s):  
Edward C. Rosenow
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Aortic Arch ◽  
Congenital Heart ◽  
Incidental Finding ◽  
Complex Congenital Heart Disease

• Right-sided aorta in an adult is almost always an incidental finding and is uncommonly associated with other anomalies • Right-sided aorta discovered in a child usually is associated with complex congenital heart disease

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