Pseudoaneurysm following modified Blalock-Taussig shunt mimicking suprasternal abscess: A rare presentation

The modified Blalock-Taussig shunt is a well-recognized palliative procedure for cyanotic congenital heart disease. Post procedure pseudoaneurysm mimicking a suprasternal abscess is of very rare occurrence. We present a child of L-Transposition of great arteries (L-TGA), pulmonary atresia and ventricular septal defect (VSD) with small pulmonary artery size that underwent modified Blalock Taussig shunt for severe cyanosis at 1 1/2 years. Six months after the procedure the child presented with a suprasternal swelling and fever. Angiography showed pseudoaneurysm from the proximal anastomotic site. The pseudoaneurysm was suitably tackled by interventional techniques and surgery for the underlying cardiac condition is being planned.     

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.     

Thrombus in Right Ventricular Outflow Tract: Unique Cause of Refractory Cyanotic Spell

Iron deficiency state in patients with cyanotic congenital heart disease can mimic as well as aggravate hyperviscosity symptoms. Correction of iron deficiency in these cases is expected to improve symptoms. We report an unexpected occurrence of refractory cyanotic spell in a child with tetralogy of Fallot due to thrombus in right ventricular outflow tract following intravenous iron sucrose therapy.     

The haematological management of patients with cyanotic congenital heart disease: A time for consensus?

AIMS: Recurrent venesection of patients with cyanotic congenital heartdisease may be detrimental, with an increased risk of cerebrovascularevents and symptomatic iron-deficiency. The aim of this studywas to determine the venesection policies as practised in hospitalswithin a U.K. region and to determine if these policies followedcurrent recommendations. METHODS AND RESULTS: Fifty-eight consultants (56% response rate) in cardiac specialtiescompleted self-assessment questionnaires regarding the indicationsfor and practice of venesection. Sixty-one percent of thoseresponding were involved directly in the care of patients withcyanotic congenital heart disease and of these clinicians 97%used venesection. Indications for venesection varied, with 51%of those responding using an elevated haemoglobin per se (6·5–21·0g.dl^–1); 78% an elevated haematocrit (0·55–0·75)and 83% symptoms. Desired maintenance haemoglobin and haematocritlevels also varied greatly. Fifty percent of the consultantsresponding routinely screened their patients for iron deficiencyand 23% felt there was no indication for investigating a lowmean corpuscular volume. Only 18% of the policies describedfollowed any evidence based principles. CONCLUSIONS: The practice of venesecting patients with congenital cyanoticheart disease varies greatly. Policies in many hospitals donot reflect the minimal benefits and considerable risks associatedwith recurrent venesection.     

Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome

Introduction

Iron deficiency is common in cyanotic congenital heart disease (CHD) and results in reduced exercise tolerance. Currently, iron replacement is advocated with limited evidence in cyanotic CHD. We investigated the safety and efficacy of iron replacement therapy in this population.

Methods

Twenty-five iron-deficient cyanotic CHD patients were prospectively studied between August 2008 and January 2009. Oral ferrous fumarate was titrated to a maximum dose of 200 mg thrice-daily. The CAMPHOR QoL questionnaire, 6 minute walk test (6MWT) and cardiopulmonary exercise testing were conducted at baseline and after 3 months of treatment.

Results

Mean age was 39.9 ± 10.9 years, 80% females. Fourteen had Eisenmenger syndrome, 6 complex cyanotic disease and 5 Fontan circulation. There were no adverse effects necessitating termination of treatment. After 3 months of treatment, hemoglobin (19.0 ± 2.9 g/dL to 20.4 ± 2.7 g/dL, p < 0.001), ferritin (13.3 ± 4.7 μg/L to 54.1 ± 24.2 μg/L, p < 0.001) and transferrin saturation (17.8 ± 9.6% to 34.8 ± 23.4%, p < 0.001) significantly increased. Significant improvements were also detected in the total CAMPHOR score (20.7 ± 10.9 to 16.2 ± 10.4, p = 0.001) and 6MWT distance (371.7 ± 84.7 m to 402.8.0 ± 74.9 m, p = 0.001). Peak VO₂ remained unchanged (40.7 ± 9.2% to 43.8 ± 12.4% of predicted, p = 0.15).

Conclusion

Three months of iron replacement therapy in iron-deficient cyanotic CHD patients was safe and resulted in significant improvement in exercise tolerance and quality of life. Identification of iron deficiency and appropriate replacement should be advocated in these patients.     

Getting to the Heart of the Matter: Epidemiology of Cyanotic Heart Defects

Congenital heart defects (CHDs) are the most common type of birth defect, making significant contributions to infant morbidity and mortality, but not all CHDs contribute equally to such outcomes. Although cyanotic CHDs constitute some of the most serious CHDs, its epidemiology is poorly understood. We present a comprehensive systematic review of the literature on the epidemiology of cyanotic CHD, with emphasis on the most current knowledge on identified risk/etiologic factors. Literature for this review was identified by searching the PubMed database from the National Center for Biotechnology Information at the US National Library of Medicine as well as bibliographies of identified papers. The 100 reports that contributed to this review describe risk factors such as infant sex, race, and ethnicity, environmental exposures, and maternal and paternal age. Several studies reported differences in prevalence rates by race and ethnicity and elevated sex ratios, and they identified some risk factors, including advanced maternal age. Investigators have made significant progress in the effort to describe the etiology of cyanotic CHDs, but discrepancies, such as the variation in prevalence rates by race and ethnicity and the impact of environmental exposures, still need to be addressed.     

Diagnosis and management of cyanotic congenital heart disease: Part II

In this review, the clinical features and management of less commonly encountered cyanotic cardiac lesions are reviewed. Pathophysiology, clinical features, laboratory studies and management are discussed. The clinical and non-invasive laboratory features of these cardiac defects are sufficiently characteristic for the diagnosis and invasive cardiac catheterization and angiographic studies are not routinely required. Such studies may be needed either to define features that could not be clearly defined by non-invasive studies or prior to performing trans-catheter interventions. Surgical correction or effective palliation is possible at relatively low risk. But, residual defects, some requiring repeat catheter or surgical intervention, may be seen in a significant percentage of patients and consequently, continued follow-up after surgery is recommended.     

Exogenous prostaglandin administration and pseudo-Bartter syndrome

Biological abnormalities simulating Bartter syndrome were observed in a preterm neonate with complex cyanotic congenital heart disease, for which ductus arteriosus was maintained open by high doses of prostaglandin (PG) until a Blalock shunt could be performed. These abnormalities spontaneously disappeared after cessation of PG administration. We postulate that the natriuretic effect of exogenous administered PG could further increase sodium wasting already induced by the cardiopathy thus leading to pseudo-Bartter syndrome.