Complex Cyanotic Congenital Heart Disease Research Articles

Mirror Mirror on the Wall: ERCP in a Patient with Situs Inversus Totalis

Situs inversus totalis is an extremely rare autosomal recessive disorder occurring in 0.01% of the population. The reversal of visceral organs poses technical difficulties for therapeutic intervention during endoscopic retrograde cholangiopancreatography (ERCP). We describe a unique case of a patient with situs inversus who underwent therapeutic ERCP for management of choledocholithiasis. A 37-year-old woman with a history of situs inversus totalis, complex congenital cyanotic heart disease, polysplenia and laparoscopic partial cholecystectomy performed 10 years prior, presented with post-prandial right upper quadrant pain and nausea. She had elevated bilirubin, and computed tomography (CT) scan showed cholelithiasis in the remnant gallbladder. An endoscopic retrograde cholangiopancreatography (ERCP) was performed for possible choledocholithiasis. Patient was placed in the left oblique position. There was altered anatomy of the gastric curvature and the ampulla was located in the 2 o'clock position. Biliary access was achieved by keeping the scope in the long position, rotating the duodenoscope 180o, and rotating the sphincterotome to the right side. Cholangiogram showed a mirror image of the transposed biliary tree and remnant gallbladder with filling defects consistent with cholelithiasis. Biliary sphincterotomy was performed and balloon sweep showed biliary stone fragments, consistent with choledocholithiasis. She subsequently underwent cholecystectomy for removal of the remnant gallbladder.Figure 1Figure 2Situs inversus is a rare benign congenital anomaly that may pose difficulty during ERCP. Different techniques have been used for biliary access in patients with situs inversus such as, a “mirror image” ERCP technique with the endoscopic maneuvers performed in an inverse fashion, or positioning the patient lying prone on her right side. In our case biliary access is easily achievable in patient s with situs inversus by keeping the patient in the left oblique position, along with maneuvering the duodenoscope and sphincterotome towards the inversed position of the ampulla.

INTIMA-MEDIA THICKNESS IS ABNORMAL IN CHILDREN WITH NON-COMPLEX CONGENITAL HEART DISEASE DESPITE SUCCESSFUL REPAIR

Children with complex cyanotic congenital heart disease (CHD) are known to be at risk for premature atherosclerosis. While little is known about the vascular status of children with repaired non-complex CHD. We have previously found evidence of oxidative stress and endothelial dysfunction in such a

Post-operative complications and measures on complex cyanotic congenital heart diseases and pulmonary shunt

Objective To analyze the causes and treatment methods of early complications after central systemic-pulmonary shunt in complex cyanotic congenital heart diseases. Methods Two hundred and twelve cases of central systemic-pulmonary shunt in complex cyanotic congenital heart diseases were retrospectively analyzed in order to explore the early postoperative complications and related treatment measures. Results There were 61 cases(28.77%) of the early postoperative complications, including severe low cardiac output syndrome in 27 cases, acute pulmonary edema in 14 cases, 24 h shunt pipe blockage in 12 cases, and supraventricular tachycardia in 8 cases.All patients got followed up, average for(2.49±1.21) years.After the systemic-to-pulmonary artery shunts, pulmonary vascular had significant growth, 8 patients(3.77%) of them who pulmonary hypoplasia were promoted by transcatheter aortopulmonary collateral vessels.At the end of the follow-up, 77 patients(36.32%)achieved the standard of radical surgery. Conclusion The factors affecting surgical survival rate include: enhancement of patients cardiac function and strictly handle operation indication before operation a clear operational view; rational surgical methods; treatment of complication without delay; strict, intensive care and synthesized treatment. Key words: Systemic-pulmonary shunt; Cardiac surgery; Complication; Congenital heart disease,

Illustrated Imaging Essay on Congenital Heart Diseases: Multimodality Approach Part III: Cyanotic Heart Diseases and Complex Congenital Anomalies.

From the stand point of radiographic analysis most of the complex cyanotic congenital heart diseases (CHD), can be divided into those associated with decreased or increased pulmonary vascularity. Combination of a specific cardiac configuration and status of lung vasculature in a clinical context allows plain film diagnosis to be predicted in some CHD. Correlation of the position of the cardiac apex in relation to the visceral situs is an important information that can be obtained from the plain film. This information helps in gathering information about the atrio-ventricular, ventricular arterial concordance or discordance. Categorization of the cyanotic heart disease based on vascularity is presented below. Thorough understanding of cardiac anatomy by different imaging methods is essential in understanding and interpreting complex cardiac disease. Basic anatomical details and background for interpretation are provided in the previous parts of this presentation.

Clinical and prognostic implications of plasma NGAL and NT-proBNP in adult patients with congenital heart disease

BackgroundPrognostic value of NT-proBNP is well established in patients with congenital heart disease. Growing evidence suggests that plasma NGAL is elevated in heart failure but data is limited in congenital heart disease.This study investigates the combined prognostic value of plasma NGAL with plasma NT-proBNP in adult patients with congenital heart disease. MethodsPlasma levels of NT-proBNP and NGAL were measured in 76 consecutive adult patients (33 men, mean age 31.7±14yrs) with congenital heart disease and normal values of serum creatinine. Patients were divided in three groups: A: simple cardiac lesions, B: complex cardiac lesions and C: cyanotic lesions. Patients were also monitored for long-term major cardiovascular events: death, hospitalization, NYHA class worsening, new onset of arrhythmias, surgical or percutaneous intervention. ResultsNGAL value was significantly different between groups: In group A median NGAL value was 64.5±36.7ng/ml, in group B median NGAL value was 88.77±36.17ng/ml and in group C median NGAL value was 121±40ng/ml (group A vs. group B: p=0.048, group B vs. group C: p=0.037, group A vs. group C: p=0.003). Plasma NT-proBNP predicted all events (HR=1.001, CI=1.001–1.002, p=0.0006) as well as cardiovascular death alone (HR=1.001, CI=1.001–1.002, p=0.0004); plasma NGAL was the only predictor of cardiovascular death (HR=1.017, CI=1.001–1.033, p=0.037). ConclusionPlasma NGAL levels were lower in patients with simple congenital disease compared to patients with complex congenital heart disease and cyanotic congenital heart disease. Plasma NGAL levels correlated with NT-proBNP and could predict cardiovascular death in this small cohort of patients.

Ischemic stroke in a child with complex cyanotic congenital heart disease

Stroke is rare in children compared to adults. Most common causes of stroke in children are heart diseases that cause embolism and cerebral infarction and arteriovenous malformations for the brain hemorrhages, but a number of cases remain idiopathic, without being able to say precise etiology. The authors present the case of a child diagnosed in infancy with complex cyanotic heart malformation, but not operated, admitted with clinical and radiological signs of pneumonia, which evolved favorably with antibiotic and symptomatic therapy. During resolution of the pneumonia, the child becomes suddenly drowsy, presenting right hemiplegia, with absence of reflexes on the same side. Clinical examination correlated with cranio-cerebral CT have established the diagnosis of bilateral Sylvian ischemic stroke. The evolution was favorable under conservative treatment (antiplatelet drugs, physiotherapy), but right hemiplegia persisted.

VACTERL rare association with rare survival pattern

Truncus Arteriosus (TA) as a cardiac anomaly is a rare occurrence in VACTERL (Vertebral defects, Anorectal malformations, Cardiac defects, Tracheo-Esophageal fistula, Renal &Limb anomalies) association and portends an extremely poor prognosis. The authors report here the case of a 37-year-old male who presented with complaints of recurrent respiratory tract infections and occasional syncopal attacks. The patient was subsequently found to possess an extremely rare combination of VACTERL association with Complex Congenital Cyanotic Heart Disease (Truncus Arteriosus with Right Sided Aortic Arch, Single Coronary Artery, Situs Inversus with Levocardia). The relatively symptom-free survival of the patient into adulthood with this combination of birth defects constitutes a medical rarity and is therefore being presented here.

ABSTRACT 809

Background and aims: Transporting critically ill children to tertiary centres capable of providing intensive care is the mainstay of pediatric emergency transportation services worldwide. Conversely, where state of the art life-sustaining interventions fail to improve outcomes and when medical futility is reached, the transfer of such patients to their homes for comfort and palliative care is seldom described in medical literature. Our Children’s Hospital Emergency Transport Service (CHETS) is a dedicated neonatal and pediatric critical care transport service established in 2004 and on average performs 90 missions annually. Our intensivists offer families palliative transfers home as part of end-of-life discussions. Aims: We report our 10 year paediatric palliative transport experience. Methods: We performed a retrospective audit of CHETS records from 2004 to 2013. Results: Eight local palliative transfers were carried out within this period. Median age of patients was 6 years (range 11 days to 14 years). Diagnoses included complex congenital cyanotic heart disease with multiorgan failure, spinal muscular atrophy Type 1 with hypoxic ischemic encephalopathy (HIE) following cardiorespiratory arrest, HIE secondary to drowning, fulminant meningoencephalitis, inoperable brainstem glioma with respiratory arrest and relapsed primitive neuroectodermal tumour with sepsis. Six patients were transported home for withdrawal of life support and two to hospices for palliation. There were no intra-transfer adverse events. Conclusions: The availability of a dedicated critical care transport service allows for palliative transfers to be carried out safely. We propose that facilitating transport to allow withdrawal of life support at home is an acceptable and valuable option to families as part of holistic end-of-life care.

Unusual osteonecrosis of the femur, tibia and fibula in an adult patient with a complex cyanotic congenital heart disease

Unusual osteonecrosis of the femur, tibia and fibula in an adult patient with a complex cyanotic congenital heart disease

Endothelial function state following repair of cyanotic congenital heart diseases

Repairing cyanotic congenital heart disease may be associated with preserving endothelial function. The present study aimed to evaluate vascular endothelial function in patients with repaired cyanotic congenital heart disease. In a case-control study conducted in 2012 in Isfahan, Iran, 42 consecutive patients aged <35 years who had suffered from different types of cyanotic congenital heart disease and had undergone complete repair of their congenital heart defect were assessed in regard to their endothelial function state by measuring flow-mediated dilatation and other cardiac function indices. They were paired with 42 sex- and age-matched healthy controls. The mean flow-mediated dilatation was lower in patients with repaired cyanotic congenital heart disease than in the controls [6.14±2.78 versus 8.16±1.49 respectively (p<0.001)]. Significant adverse correlations were found between flow-mediated dilatation, age, and body mass indexes, in those who underwent repair surgery. In addition, flow-mediated dilatation had a positive association with the shortening fraction, ejection fraction, and tricuspid annular plane systolic excursion value, and it was also inversely associated with carotid intima-media thickness and the myocardial performance index. The mean of the flow-mediated dilatation was significantly higher in the group with tetralogy of Fallot along with complete repair before the age of 2.5 years and also in those patients with total anomalous pulmonary venous connection or transposition of the great arteries repaired with an arterial switch operation before 6 months of age, compared with the other two subgroups. This includes patients with a tetralogy of Fallot defect repaired after 4 years of age and those with complex cyanotic congenital heart disease that was repaired after 2.5 years of age (mean age at repair 9±6.1 years). Early repair of a cyanotic defect can result in the protection of vascular endothelial function and prevent the occurrence of vascular accidents at an older age.

“Downhill” Esophageal Varices in Congenital Heart Disease

“Downhill” Esophageal Varices in Congenital Heart Disease

Extended Criteria for Organ Acceptance Following Paediatric Donation after Circulatory Death (DCD): An Underdeveloped Potential

Introduction: There are more patients waiting for organ transplantation than there are available donor organs. In 2008, the UK commissioned an Organ Donation Taskforce to identify obstacles to successful organ transplantation [1]. National initiatives implement the recommendations: the consideration of organ donation is to become standard within end-of-life care. The diagnosis of brain stem death in paediatrics is unusual. In hospital, children may die following controlled withdrawal of life sustaining support. Organ retrieval in paediatrics is more reliant upon donation after circulatory death (DCD). The paediatric DCD program is innovative[2]; the ethical justification of change in clinical practice to accommodate the intended benefit of organ optimisation is controversial and the desired clinical parameters are not clearly defined. Clinical experience and expertise is limited. We report our first and the UK's youngest and smallest DCD that successfully progressed to transplantation. History: Our patient was a 7month 7kg infant with hypoplastic left heart syndrome (HLHS) - complex cyanotic congenital heart disease (CyCHD) where the systemic and pulmonary circulations are in parallel and result in an inherently unstable hypoxic systemic blood flow. The infant died 55 days after her 4th admission to PICU following a series of complex palliative cardiac interventions (Glenn operation and cardiac catheterisations). Our patient was dependent upon continuous inotropic support (milrinone and adrenaline), almost exclusively ventilated and had saturations 65-75%. There was clinical suspicion of chronic global ischaemia (difference in arterio-venous saturation 40-45%). The infant had been considered a candidate for heart transplantation. During end-of-life discussions, the parent's were keen to explore organ donation. The liver was declined by all UK transplanting centres due to function or size. A European centre provisionally accepted, pending direct visualisation. Only one UK centre expressed an interest in the kidneys in view of the generalised lack of experience of implanting paediatric DCD kidneys. Results: The patient became asystolic within 23 minutes of withdrawal of support. Cold aortic perfusion commenced 12 minutes thereafter. The liver was considered unfavourable. The kidneys were transplanted en-bloc into a haemodialysis dependent adult.Table: [Recipient Renal Function]Conclusion: Our case illustrates that successful transplantation following paediatric DCD is possible, despite the ‘unfavourable’ donor haemodynamic profile and the real-ime pragmatic unease imposed by the novel clinical practice changes. Transplanted infant kidneys, previously exposed to chronic marginal hypoxia and ischaemia, have the potential to normalise an adult's biochemistry. Paediatric specific threshold clinical parameters considered acceptable to explore organ transplantation have not been qualified. This allows variation in practice and bias towards excessive restriction jeopardizing the fate of potentially transplantable organs. Adult parameters cannot be applied to the child, especially if the deceased child suffered from cyanotic CHD. Clarification is needed to standardize practice and prevent the emergence of unsubstantiated obstacles. Acknowledgement: The authors acknowledge the exceptional courage and generosity of the donor family involved.

A case of single atrium and single ventricle physiology with bilateral cleft lip and palate for lip repair surgery

Bilateral cleft lip and palate may occasionally be associated with complex congenital cyanotic heart disease. An infant with common atrium and single ventricle with infundibular pulmonary stenosis (Blalock-Taussig shunt done recently) presented for lip repair surgery. Balanced general anesthesia was administered using sevoflurane along with a regional nerve block to maintain optimal pulmonary and systemic vascular resistance.

Headache and Papilledema in an Adult with Cyanotic Congenital Heart Disease: The Importance of Fundoscopic Evaluation Rather Than Phlebotomy

Headaches and blurred vision in patients with cyanotic congenital heart disease and secondary erythrocytosis may be attributed to hyperviscosity and traditionally were treated with phlebotomy. In the current era, phlebotomy is rarely performed in these patients except in cases of hemoptysis or hyperviscosity symptoms. We report a case of a patient with a history of complex cyanotic congenital heart disease and secondary erythrocytosis who presented with headache and visual changes. He was found to have bilateral papilledema and increased intracranial pressure. Reduction of intracranial pressure with acetazolamide therapy led to alleviation of headache and visual changes. This demonstrates the need for formal ophthalmologic evaluation of these patients to assess other treatable causes of headache and visual changes before considering phlebotomy.

Transient enhancement of pericardium, peritoneum, soft tissues and perhaps lymphatics after large doses of contrast administration: an overlooked phenomenon?

An infant with complex cyanotic congenital heart disease was recently encountered whose radiographs seemed to show enhancement of pericardium, peritoneal mesothelium and body wall fascial planes without enhancement of the liver or spleen after very large doses of intravenous contrast. Although patterns of postcontrast enhancement have been described previously, this pattern seems to be unique. We report the unusual postcontrast opacification pattern and speculate about its underlying mechanism.

Gadolinium-Enhanced Three-Dimensional Magnetic Resonance Angiographic Assessment of the Pulmonary Artery Anatomy in Cyanotic Congenital Heart Disease With Pulmonary Stenosis or Atresia: Comparison With Cineangiography

Pulmonary stenosis (PS) or pulmonary atresia (PA) is an important component of complex cyanotic congenital heart disease, especially in tetralogy of Fallot or lesions with ventricular septal defect (VSD)-PS physiology. Management strategy in these patients depends on accurate assessment of PAs and identification of all sources of pulmonary blood flow. X-ray cineangiography is the "gold standard" for this purpose, but it has the inherent risks of an invasive procedure. Gadolinium-enhanced three-dimensional magnetic resonance angiography (Gd-MRA) has been shown to noninvasively and accurately evaluate various lesions of the vascular system. This study was undertaken to evaluate the accuracy of Gd-MRA compared with cineangiography in the evaluation of pulmonary anatomy. Nineteen patients having complex cyanotic heart disease with PS or PA were included in the study. All patients underwent Gd-MRA and cineangiography. Catheterisation and MRA findings regarding the anatomic variable of interest were analysed for agreement by Bland-Altman analysis. There was total agreement between the two modalities in the delineation of confluent PAs. McGoon's ratio and the Nakata index, which are standard measures of the adequacy of PA size, also showed excellent agreement between the two modalities. MRA was able to delineate all aorto-pulmonary collaterals in the setting of PA. MRA can delineate all sources of pulmonary blood supply in cyanotic congenital heart disease with PS and/or PA as well as provide accurate assessment of PA size for planning corrective surgery.

Understanding the physiology of complex congenital heart disease using cardiac magnetic resonance imaging

Complex congenital heart diseases are often associated with complex alterations in hemodynamics. Understanding these key hemodynamic changes is critical to making management decisions including surgery and postoperative management. Existing tools for imaging and hemodynamic assessment like echocardiography, computed tomography and cardiac catheterization have inherent limitations. Cardiac magnetic resonance imaging (MRI) is emerging as a powerful bouquet of tools that allow not only excellent imaging, but also a unique insight into hemodynamics. This article introduces the reader to cardiac MRI and its utility through the clinical example of a child with a complex congenital cyanotic heart disease.

Infective endocarditis complicated by fatal cerebral hemorrhage revealing an unrepaired univentricular heart in a 19‐year‐old woman

Univentricular heart is a complex and rare cyanotic congenital heart disease. When not operated, affected patients exceptionally reach adulthood. We report the unprecedented case of a 19 year-old young woman, admitted to the hospital for a severe deterioration of general status and ultimately diagnosed to have an infective endocarditis with multiple vegetations in a previously undiagnosed univentricular heart of left ventricular morphology, subsequently rapidly complicated by fatal cerebral hemorrhage.

Stent Implantation of the Arterial Duct in Newborns with a Truly Duct‐Dependent Pulmonary Circulation: A Single‐Center Experience with Emphasis on Aspects of the Interventional Technique

Ductal stenting for pulmonary blood supply in newborns with cyanotic congenital heart disease (CHD) might be a low risk and safe alternative to the surgical aorto-to-pulmonary artery (AP) shunt in dual-source lung perfusion. Ductal stenting in truly duct-dependent pulmonary circulation has not been evaluated. Prospective interventional and clinical follow-up trial. Ductal stenting based on variable access sites, a 2-wire technique when crossing a tortuous ductus, and use of premounted coronary stents. Primary outcome measures were procedural success and complication rates presented as early and mid-term results. From 2003-2009, 58 duct-dependent newborns underwent ductal stenting; 27 of them were truly duct dependent, 20 had pulmonary atresia (PA)/ventricular septum defect or complex CHD, 4 had PA/intact ventricular septum, 2 had PA with Ebstein anomaly, and 1 had PA with tricuspid atresia. Ductal stenting was performed without procedure-related mortality; 3 of 27 required an acute surgical AP-shunt (stent migration in 1, acute duct obstruction in 2). During mid-term follow-up, 4 of 24 needed an AP-shunt and two others stent redilation. Three patients died prior to follow-up surgery (1 unexpectedly at home and 2 due to syndromatic disease). Fifteen patients received staged univentricular palliation, 8 had a biventricular repair, and 1 is awaiting follow-up operation. Ductal stenting is a feasible, safe, and effective palliation in newborns with truly duct-dependent pulmonary circulation irrespective of duct morphology. Vasucular access from various locations is important for technical success rate. Ductal stenting is a minimally invasive procedure to achieve adequate pulmonary artery growth for subsequent palliative or corrective surgery.

MRI of complex cyanotic congenital heart disease: pre- and post surgical considerations

Evaluation of patients with complex congenital cyanotic heart disease requires a solid knowledge base of the underlying defects and a full understanding of surgical palliative and corrective procedures. As these patients survive into adulthood, understanding common conditions, their associated surgical procedures, and potential surgical complications is paramount for the radiologist. Use of magnetic resonance imaging in evaluation of these patients is a critical tool at the disposal of the radiologist.