Novel transcatheter intervention for iatrogenic cyanosis due to surgical rerouting of inferior vena cava to the left atrium.

Improper identification of the atrial septal defect margins during surgery and inadvertent suturing of the surgical patch to the Eustachian valve of the inferior vena cava (IVC) results in the diversion of inferior venacaval blood to the left atrium causing cyanosis. This complication has been dealt so far with surgery. We report the planning and implementation of a novel transcatheter rediversion of the IVC to the right atrium using a covered stent.

MRI characterization of hemodynamic patterns of human fetuses with cyanotic congenital heart disease.

OBJECTIVES: To characterize, using magnetic resonance imaging (MRI), the distribution of blood flow and oxygen transport in human fetuses with subtypes of congenital heart disease (CHD) that present with neonatal cyanosis. METHODS: Blood flow was measured in the major vessels of 152 late-gestation human fetuses with CHD and 40 gestational-age-matched normal fetuses, using cine phase-contrast MRI. Oxygen saturation (SaO2 ) was measured in the major vessels of 57 fetuses with CHD and 40 controls. RESULTS: Compared with controls, we found lower combined ventricular output in fetuses with single-ventricle physiology, with the lowest being observed in fetuses with severe forms of Ebstein's anomaly. Obstructive lesions of the left or right heart were associated with increased flow across the contralateral side. Pulmonary blood flow was reduced in fetuses with Ebstein's anomaly, while those with Ebstein's anomaly and tricuspid atresia had reduced umbilical flow. Flow in the superior vena cava was elevated in fetuses with transposition of the great arteries, normal in fetuses with hypoplastic left heart, tetralogy of Fallot or tricuspid atresia and reduced in fetuses with Ebstein's anomaly. Umbilical vein SaO2 was reduced in fetuses with hypoplastic left heart or tetralogy of Fallot. Ascending aorta and superior vena cava SaO2 were reduced in nearly all CHD subtypes. CONCLUSIONS: Fetuses with cyanotic CHD exhibit profound changes in the distribution of blood flow and oxygen transport, which result in changes in cerebral, pulmonary and placental blood flow and oxygenation. These alterations of fetal circulatory physiology may influence the neonatal course and help account for abnormalities of prenatal growth and development that have been described in newborns with cyanotic CHD. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Low threshold for intracranial imaging in fever of unknown origin associated with cyanotic heart disease in the pediatric population.

Intracranial abscess in the pediatric population is an overall rare occurrence-4 in a million. The most common predisposing factor is underlying cyanotic congenital heart disease (CCHD), which is associated with ~ 30% of all cases. We present an unusual case of cerebral abscess in a 17-month-old female with partially treated Tetralogy of Fallot and fever of unknown origin without associated neurologic symptoms. We propose a low threshold for intracranial imaging as part of the fever of unknown origin work-up in children with underlying cyanotic congenital heart disease.

Prenatal Growth in Fetuses with Isolated Cyanotic and Non-Cyanotic Congenital Heart Defects.

BACKGROUND: Fetal growth may vary significantly in different congenital heart defects (CHDs). OBJECTIVES: To investigate prenatal growth of CHD fetuses and its correlation with classifications based upon expected oxygen delivery to the fetal brain or structural findings. METHODS: Seventy-nine euploid fetuses with isolated CHD were recruited prospectively and categorized by the expected oxygen supply to the brain (low, intermediate, and high) or by the expected arterial mixing considering two categories (cyanotic or non-cyanotic). Biometry and Doppler were recorded, and Z-scores (Zs) calculated. Growth changes at different time points were analyzed and compared with 150 controls. RESULTS: A total of 664 exams were performed on 229 fetuses. Median head circumference (HC) Zs were lower in all CHD fetuses from the second trimester onwards and in cyanotic CHD fetuses from the first onwards, with associated smaller abdominal circumference (AC) in the third trimester (first-trimester biparietal diameter Zs cyanotic: -1.3 [-2.36; -0.98], non-cyanotic -0.72 [-1.25; -0.6], p = 0.044, second-trimester HC Zs cyanotic: -1.47 [-2.3; -0.84]; non-cyanotic -0.45 [-0.83; -0.02], p < 0.0001; AC Zs cyanotic 0.0 [-0.44; 0.86]; non-cyanotic 0.65 [0.31; 1], p = 0.0006). Birth-weight centiles were smaller in CHDs (particularly in cyanotic) with no differences between categories of brain oxygen delivery. CONCLUSIONS: Fetuses with cyanotic CHD have fetal growth restriction, impaired head growth, yet normal posterior fossa dimensions and fetal-placental Doppler.

Cyanotic Congenital Heart Disease Modes of Presentation and Prenatal Detection

Prenatal detection of structural congenital heart disease (CHD) optimises cardiovascular stability pre-operatively and post-operative outcomes. We compared prenatal detection rates of critical CHD in units offering universal fetal anomaly scans with those offering imaging to selected women. One hundred and thirteen infants met inclusion criteria. The overall pre-natal detection rate for critical CHD was 57% of liveborn infants. It was 71% (57/80) in hospitals who offered a universal anomaly scan and 29% (9/31) in centres offering a limited service. Postnatal diagnosis was associated with PICU admission (p=0.016) and preoperative mechanical ventilation (p=0.001). One-year mortality was 10 fold higher in the postnatally diagnosed group 15% vs 1.55% (p=0.0066). There is a significant disparity between centres offering universal anomaly versus selective screening. Prenatal detection confers advantage in terms of pre-operative stability and one year survival. Failure to deliver an equitable service exposes infants with CHD to avoidable risk.

Hippocampal damage and memory impairment in congenital cyanotic heart disease.

Neonatal hypoxia can lead to hippocampal atrophy, which can lead, in turn, to memory impairment. To test the generalizability of this causal sequence, we examined a cohort of 41 children aged 8-16, who, having received the arterial switch operation to correct for transposition of the great arteries, had sustained significant neonatal cyanosis but were otherwise neurodevelopmentally normal. As predicted, the cohort had significant bilateral reduction of hippocampal volumes relative to the volumes of 64 normal controls. They also had significant, yet selective, impairment of episodic memory as measured by standard tests of memory, despite relatively normal levels of intelligence, academic attainment, and verbal fluency. Across the cohort, degree of memory impairment was correlated with degree of hippocampal atrophy suggesting that even as early as neonatal life no other structure can fully compensate for hippocampal injury and its special role in serving episodic long term memory. © 2017 Wiley Periodicals, Inc.

Late desaturation due to giant azygous vein 16 years after bidirectional cavopulmonary anastomosis.

We present a rare late manifestation of systemic venous collaterals in a 17-year-old female, 16 years after bidirectional cavopulmonary anastomosis, resulting in clinically unacceptable desaturation with progressive effort intolerance and cyanosis.

Echocardiographic differential diagnosis of the cyanotic newborn.

Cyanosis in newborns can be caused by cyanotic heart defects, such as transposition of the great arteries, tetralogy of Fallot, pulmonary and tricuspid atresia, hypoplastic left heart syndrome, common arterial trunk, Ebstein's anomaly of the tricuspid valve, and total anomalous pulmonary venous return. The indicated cyanotic heart defects can be diagnosed or ruled out with three simple echocardiographic views: The parasternal long and short axis view and the apical or subcostal 4-chamber view. If these three views are normal, a cyanotic heart defect can be ruled out. In the case of a cyanotic heart defect, one or more views are pathological.

Evaluation of depression and anxiety in parents of children undergoing cardiac catheterization.

OBJECTIVE: This study aimed to determine pre-procedure depression and anxiety levels among a group of parents whose children had congenital heart disease and were undergoing angiography. METHODS: The study comprised parents of 73 congenital heart disease patients undergoing angiography. The Beck Depression Inventory (BDI) and the Beck Anxiety Inventory (BAI) were used to evaluate the depression and anxiety scores. RESULTS: Sixty-one patients (83.6%) had acyanotic congenital heart disease, and 25 patients (34.2%) were undergoing diagnostic angiography. BDI scores among the mothers determined that 8 (11%) had mild, 14 (19.2%) moderate, and 10 (13.7%) severe depression. Their BAI scores showed that 16 (21.9%) had mild, 8 (11%) moderate, and 13 (17.8%) severe anxiety. BDI scores for the fathers showed that 12 (16.4%) had mild, 10 (13.7%) moderate, and 8 (11%) severe depression. Their BAI scores showed that 12 (16.4%) had mild, 10 (13.7%) moderate, and 8 (11%) severe anxiety. A comparison of mothers of cyanotic patients and those of acyanotic patients in terms of depression and anxiety levels revealed a statistically significant difference (p=0.050 and 0.043, respectively). CONCLUSION: Angiography was associated with increased levels of depression and anxiety in parents of children with congenital heart diseases. In comparison to parents of patients with acyanotic congenital heart disease, mothers of patients with cyanotic congenital heart disease had significantly higher levels of depression and anxiety.

Cyanosis in a 9-month-old child after repair of total anomalous pulmonary venous return.

A 9-month-old girl presented with cyanosis after correction of total anomalous pulmonary venous return (TAPVR) to the coronary sinus in the neonatal period. During corrective surgery, the right superior vena cava (RSVC) was damaged and re-anastomosed to the right atrium. Echocardiography showed increased flow velocity in the pulmonary venous confluence. Therefore, pulmonary venous obstruction was suspected. However, subsequent cardiac MRI revealed a stenosed RSVC as well as a dilated left superior vena cava (LSVC) draining from the left innominate vein into the pulmonary venous confluence. The re-recruited LSVC drained deoxygenated blood into the systemic circulation, causing cyanosis. After uncomplicated placement of a stent in the narrowed RSVC and occlusion of the LSVC, transcutaneous saturations normalised immediately.

An unusual cause of central cyanosis in a nine-year-old boy.

Pulmonary arteriovenous malformations are rare vascular anomalies which rarely present in childhood. We discuss a nine-year-old male child who presented with the classical triad of exertional dyspnea, cyanosis and clubbing since six years of age. Pulmonary arteriovenous malformation was suspected on chest radiography and was later confirmed on computed tomography scan. A bubble contrast echocardiography showed the presence of an extracardiac shunt. Pulmonary angiography was done to delineate the anatomy of the pulmonary vasculature and to plan embolization. The child has undergone two sittings of transcatheter coil embolization with improvement in oxygenation.

Systemic venous collateral channel causing desaturation after bidirectional cavopulmonary anastomosis: percutaneous closure.

An infant with cyanotic cardiac disease that was palliated with a bidirectional cavopulmonary shunt developed progressive and worsening cyanosis 5 years after the surgical procedure. A large venous collateral was found to be decompressing the bidirectional Glenn shunt from the superior caval vein to the inferior caval vein and was percutaneously closed with a vascular plug. The unusually large venous collateral, and the excellent outcome associated with percutaneous procedure are discussed.

Ebstein's anomaly in pregnancy: maternal and neonatal outcomes.

AIM: Ebstein's anomaly is a rare congenital cardiac abnormality, associated with cyanosis and arrhythmia. Patients often reach childbearing age and pregnant women pose a challenge to the treating physician. We describe the course and outcome of pregnancy in women with Ebstein's anomaly and discuss the related management issues. METHODS: Analysis of pregnancies in four women with Ebstein's anomaly was carried out in a referral institute in northern India. Data of two women were analyzed retrospectively and the other two women were prospectively followed in their pregnancies during the study period. The course of the pregnancy, disease and perinatal outcome in each woman were analyzed. RESULTS: Four women had eight pregnancies all resulting in vaginal deliveries. There were two premature deliveries. The mean birth weight was 2.54 + or - 0.88 kg. Of the eight babies, six did not have any cardiac anomalies; one was an unexplained neonatal death and for another, no data was available. Pregnancy was well tolerated in two patients, while one had right heart failure during early pregnancy, and one had arrhythmia during labor, which was managed medically; one patient had arrhythmia in the postpartum period, which was managed medically. CONCLUSION: When a woman with Ebstein's anomaly reaches childbearing age, fertility is not affected, even in cyanotic women. Under close supervision by the woman's obstetrician and cardiologist, the pregnancy outcome is usually favorable.

A novel hybrid method for creating a porcine model of cyanotic congenital heart defect with decreased pulmonary blood flow.

OBJECTIVE: To create an experimental model of cyanotic congenital heart defect with decreased pulmonary blood flow using a novel hybrid method. METHODS: A model of congenital heart defect with decreased pulmonary blood flow and chronic cyanosis was produced surgically in eight piglets (mean, 2 mo old). An artificial atrial septal defect was created followed by pulmonary artery banding to generate a systolic pressure gradient at 2 mo of > or = 50 mm Hg (cyanotic group). Another eight piglets underwent a sham operation (control group). RESULTS: There were six long-term survivors in the cyanotic group. At a mean duration of 2 mo following the hybrid procedure, the resting gradient across the pulmonary artery band was 53.7 mm Hg. The Qp/Qs reached 0.54:1. The arterial oxygen tension, arterial oxygen saturation, hematocrit, and hemoglobin concentration were 52.9 mm Hg, 85.6%, 49.8%, and 16.6 g/dL in the cyanotic group versus 118.0 mm Hg, 98.0%, 37.9%, and 12.2 g/dL in the control group, respectively (all P < 0.001). CONCLUSIONS: A porcine model of cyanotic congenital heart defect with decreased pulmonary blood flow was established by a hybrid method. Application of this experimental design may enhance our understanding and possibly influence the treatment of patients who have cyanotic heart disease with decreased pulmonary blood flow.

Fatal Myocarditis in an Adolescent Girl with Evolving Connective Tissue Disease.

Connective tissue diseases are rarely suspected and diagnosed in childhood and adolescence. Rarity of occurrence and poor disease acceptance among parents make them extremely difficult to treat in the early stages. An adolescent girl presented with features of pneumonia, was worked up and diagnosed as an evolving connective tissue disease. Her clinical characteristics did not fit into any specific disease. She was started on steroids and immunoglobulin as she had fulminant myocarditis with rapid downhill clinical course. High index of suspicion and aggressive immunosuppression can be life saving in exceptional situations even though a specific diagnosis cannot be ascertained.

Subclinical atherosclerosis in patients with cyanotic congenital heart disease.

INTRODUCTION: Survival in patients with cyanotic congenital heart disease (CCHD) has improved dramatically. The result is an ageing population with risk of acquired heart disease. Previous small uncontrolled studies suggested that these patients are protected against the development of atherosclerosis. To test this hypothesis, we sought to determine the prevalence of subclinical atherosclerosis in a larger population of patients with CCHD. METHOD: We compared the prevalence of subclinical atherosclerosis in adult CCHD patients from Denmark, Sweden, Norway and Australia, with that in age-, sex-, smoking status-, and body mass index matched controls. Coronary artery atherosclerosis was assessed on computed tomography with coronary artery calcification (CAC) score. Subclinical atherosclerosis was defined by CAC-score > 0. Carotid artery atherosclerosis was evaluated using ultrasound by measuring carotid plaque thickness (cPT-max) and carotid intima media thickness (CIMT). Lipid status was evaluated as an important atherosclerotic risk factor. RESULTS: Seventy-four patients with CCHD (57% women, median age 49.5 years) and 74 matched controls (57% women, median age 50.0 years) were included. There were no differences between the groups in: CAC-score > 0 (21% vs. 19%, respectively; p = 0.8), carotid plaques (19% vs. 9%, respectively; p = 0.1), cPT-max (2.3 mm vs. 2.8 mm, respectively; p = 0.1) or CIMT (0.61 mm vs. 0.61 mm, respectively; p = 0.98). And further no significant differences in lipoprotein concentrations measured by ultracentrifugation. CONCLUSION: Young adults with CCHD have similar cardiovascular risk factor profiles and measures of subclinical atherosclerosis, compared with controls. Given their increasing life expectancies, athero-preventive strategies should be an important part of their clinical management.

Amplatzer vascular plug closure of a Blalock-Taussig shunt through a Glenn shunt.

We describe an unusual route and uncommon device use for the percutaneous closure of a modified Blalock-Taussig (BT) shunt. A 4-year-old male had presented with severe upper body swelling after a bidirectional Glenn shunt done one month earlier. The BT shunt which was clipped during the surgery was found to be patent. The BT shunt was finally closed from the bidirectional Glenn using an Amplatzer vascular plug, resulting in symptom relief.

Percutaneous management of cyanosis in Fontan patients using Amplatzer occluders.

OBJECTIVES: To determine causes of cyanosis and to evaluate percutaneous management of cyanosis in a group of consecutive Fontan patients. BACKGROUND: A variety of communications allow a right-to-left shunt in Fontan circulation causing cyanosis and these communications are amenable to percutaneous closure. METHODS: Between November 1997 and November 2007, 45 consecutive patients ranging in age from 2.5 to 26 years (median 8 years) with Fontan circulation and cyanosis underwent cardiac catheterization and percutaneous closure of superfluous communications using different types of Amplatzer occluders. RESULTS: Altogether, 51 communications were detected and 50 of them were closed. In 5 patients (11.1%), multiple communications were present and were closed. Fenestration was detected in 37 of 41 patients (90.2%) after total cavopulmonary connection and all were closed by Amplatzer septal occluders. Five venous collaterals were revealed in 3 of 41 patients (7.3%) with total cavopulmonary anastomosis and were closed by Amplatzer vascular plugs. In 3 patients, lateral tunnel leaks were detected and were closed by Amplatzer PFO occluders. In 4 patients after Kawashima operation, 3 major pulmonary arterio-venous malformations and single venous collateral contributed to the cyanosis and all were closed using Amplatzer vascular plugs. CONCLUSIONS: Different communications cause cyanosis in Fontan patients. Progressive decline of percutaneous oxygen saturation is suggestive of development of venous collaterals or pulmonary arteriovenous malformations. Superfluous communications are amenable to percutaneous closure using various types of Amplatzer occluders. A novel use of an Amplatzer PFO occluder for the percutaneous closure of a lateral tunnel leak is described.