Elizabeth Orchard

Partial Congenital Absence of the Pericardium

A 45-year-old male was admitted to the hospital with recurrent, stabbing, nonexertional chest pain. Chest x-ray showed an increased cardiothoracic ratio (Figure 1A), and ECG demonstrated sinus rhythm with incomplete right bundle-branch block and poor R-wave progression (Figure 1B). Echocardiography showed normal left ventricular dimensions and function; however, the right ventricle appeared grossly dilated with moderate to severe tricuspid regurgitation with no evidence of intracardiac shunts. These findings were confirmed by right heart catheterization, which also showed normal pulmonary and right atrial pressures without a pressure gradient over the pulmonic valve. Catheterization of the left heart showed normal ventricular pressures and function as well as normal coronary arteries. The patient was referred for cardiac magnetic resonance imaging (CMR) with suspected right ventricular cardiomyopathy. CMR showed cardiac displacement into the left hemithorax (Figure 2A). There was severe tricuspid regurgitation with marked dilatation of both the right atrium (6×8 cm) and ventricle (end-diastolic volume, 343 mL). Right ventricular function was mildly reduced (ejection fraction, 46%) without localized wall motion abnormalities. Left ventricular volumes and …

Prominence of the Eustachian valve in paradoxical embolism

AIMS to investigate the relationship between Eustachian valve (EV) length and degree of atrial septal movement in patients with patent foramen ovale (PFO) and presumed paradoxical cerebral embolism. PFO is a well-established risk factor for cryptogenic stroke. However, due to the high prevalence of PFO, many of these are bystanders rather than true pathological entities. Other studies have sought to define which patients with PFO are particularly at risk of cryptogenic stroke by measuring various parameters of right atrial anatomy. We investigated the relationship between EV length and atrial septal movement. METHODS AND RESULTS measurements of EV length and atrial septal movement were made prospectively from 72 consecutive patients referred to our centre for PFO closure following presumed cryptogenic stroke, by intracardiac phased array echocardiography. The most significant finding from this study was that patients with fewer than 10 mm atrial septal movement had significantly longer EVs than those in whom there was >10 mm septal movement (P = 0.003). The mean EV length with >10 mm septal movement is 6.35 mm, and 13.33 mm with fewer than 10 mm movement. The prevalence of septal movement beyond 10 mm was significantly less in our series than in previously published papers. CONCLUSION we propose that while a large degree of atrial septal movement significantly increases propensity to cerebral embolism in patients with PFO, its absence does not negate this risk. We have shown that long EV may function independently from atrial septal movement to potentiate paradoxical embolism.

Congenitally Corrected Transposition of the Great Arteries Presenting in a Nonagenarian

An 82-year-old woman presented with recurrent syncope. Clinically, she had a slow rising pulse, right parasternal heave, and an ejection systolic murmur loudest at the right sternal edge with a quiet pansystolic murmur at the apex. ECG demonstrated prolonged PR interval and extreme leftward axis. Transthoracic and transesophageal echocardiography revealed atrioventricular and ventriculoarterial discordance, suggesting congenitally corrected transposition of the great arteries (ccTGA). The systemic right ventricle (RV) was hypertrophied and mildly dilated with normal systolic function, but there was severe subvalvular stenosis with a peak velocity of 4.5 m/s (Figures 1 and 2⇓). The aortic valve was trileaflet and anterior to the pulmonary valve, and there was mild left atrioventricular valve regurgitation. Figure 1. Transthoracic echocardiographic images. Shown are representative images from transthoracic echocardiography at …

Endovascular stent placement is an acceptable alternative to reoperation in selected infants with residual or recurrent aortic arch obstruction

Objective: To describe endovascular stent placement in infants as a technically feasible option in circumstances where surgery is considered less favorable. Background: Endovascular stent placement has become established as a first line therapy for native coarctation of the aorta or recoarctation in older children where stents capable of expansion to adult size can be placed safely. Surgery remains the therapy of choice in infants and young children. The management of aortic arch obstruction in infants is, however, frequently complicated by complex anatomy or clinical condition that may make surgery or further surgery an unattractive option. There is little reported data and the implications thereof of transcatheter stent placement in aortic arch obstruction in infants. Methods: Between August 2004 and November 2009, 11 patients had aortic arch obstruction treated with endovascular stent placement. The median age and weight at first stent placement was 46 days (range 3–399 days) and 4 kg (range 1.4–8 kg), respectively. In 10 patients, surgical intervention preceded transcatheter stent placement. Four had complex aortic arch obstruction and seven had recoarctation. Results: Reduction in peak systolic gradient to <10 mm Hg was achieved in seven of 10 patients with an improvement in aortic artery diameter to >90% of adjacent aorta in all. The diameter of the arch obstruction increased from a median of 1.60 to 4.90 mm (P = 0.001) and the peak systolic gradient from 45 mm Hg to 8 mm Hg (P < 0.0001). Adverse events occurred in two patients one who required further surgical revision and a second who required placement of a second stent. The median follow up was 3.60 years (range 0.4–5.5 years) with two patients having died at 1.34 and 1.42 years poststent placement. Of the nine patients alive, six have since undergone further angioplasty at a median time interval of 0.77 years (range 0.17–2.76 years). Long‐term complications occurred in none. Conclusions: Endovascular stent placement in infants is technically feasible with good results achievable even in small babies. It should be considered as a therapeutic option in complex cases when surgical alternatives are less favorable.

Reducing ionizing radiation doses during cardiac interventions in pregnant women

Background There is concern over ionizing radiation exposure in women who are pregnant or of child-bearing age. Due to the increasing prevalence of congenital and acquired heart disease, the number of women who require cardiac interventions during pregnancy has increased. We have developed protocols for cardiac interventions in pregnant women and women of child-bearing age, aimed at substantially reducing both fluoroscopy duration and radiation doses. Methods Over five years, we performed cardiac interventions on 15 pregnant women, nine postpartum women and four as part of prepregnancy assessment. Fluoroscopy times were minimized by simultaneous use of intracardiac echocardiography, and by using very low frame rates (2/second) during fluoroscopy. Results The procedures most commonly undertaken were closure of atrial septal defect (ASD) or patent foramen ovale (PFO) in 16 women, coronary angiograms in seven, right and left heart catheters in three and two stent placements. The mean screening time for all patients was 2.38 minutes (range 0.48–13.7), the median radiation dose was 66 (8.9–1501) Gy/cm2. The median radiation dose to uterus was 1.92 (0.59–5.47) μGy, and the patient estimated dose was 0.24 (0.095–0.80) mSv. Conclusions Ionizing radiation can be used safely in the management of severe cardiac structural disease in pregnancy, with very low ionizing radiation dose to the mother and extremely low exposure to the fetus. With experience, ionizing radiation doses at our institution have been reduced.

Niemann-Pick disease type B with severe coronary artery disease and early recurrence after coronary artery bypass grafting

A Caucasian female presented at age 50 years with severe increasing exertional angina. Her only risk factor for ischemic heart disease was an adverse fasting lipid profile: total cholesterol 7.2 mmol/L (277.2 mg/dL), high-density lipoprotein cholesterol (HDL-C) 0.5 mmol/L (19.2 mg/dL), low-density lipoprotein cholesterol (LDL-C) 5.5 mmol/L (212.7 mg/dL), and triglycerides 2.7 mmol/L (239.22 mg/ dL). She was a nonsmoker, normotensive, and nondiabetic, with no family history of ischemic heart disease. The patient was known to have Niemann-Pick disease type B. She presented with hepatosplenomegaly at the age of 2, and at age 33 years the diagnosis was suggested by examination of a bone marrow aspirate (visualized with May-Grunwald-Giemsa stain) containing histiocytic cells laden with blue and green granular material (‘‘sea blue histiocytes’’). The diagnosis was confirmed on finding a low activity of the leukocyte acid sphingomyelinase of 0.35 nmol/hour/mg protein (normal range 0.86–2.8). Coronary angiography demonstrated total occlusion of the right coronary artery with a critical stenosis in the proximal left anterior descending artery. Left ventricular angiography revealed mild anterior hypokinesis with moderate reduction in overall left ventricular function. She

Fungal myocarditis in a preterm neonate

A male infant born at 25 weeks gestation presented with abdominal distension, was transferred to our institution for surgical management following suspected bowel perforation with severe sepsis. Umbilical catheter cultures grew Candida parapsilosis. At laparotomy, there was a large ileal perforation with peritonitis, he was treated with amphotericin, antibiotics and had an ileostomy. He had persistent pulmonary hypertension, requiring nitric oxide and high-frequency oscillatory ventilation. Serial echocardiograms revealed a patent ductus arteriosus (PDA), but also demonstrated increasing left ventricular hypertrophy and the development of bright areas within the septal myocardium. Further bright areas developed over a course of 2 weeks in his right ventricular outflow tract. After treatment for candidal infection, there was improvement in left ventricular thickness and brightness of the echogenic lesions was reduced. Biopsy of the lesions was discounted due to the risk of the procedure, the size of the infant and his improving clinical status.

The management of cryptogenic stroke in pregnancy

Cerebrovascular accidents (CVAs) during pregnancy are uncommon but can have devastating consequences. The causes of CVA in both the pregnant and the non-pregnant state are diverse and require thorough investigation. Recent studies have indicated that embolic stroke in young adults may be caused by paradoxical emboli through a patent foramen ovale (PFO), suggesting that the presence of a PFO should be specifically sought in pregnant or postpartum women presenting with CVA. This review will outline the causes of CVAs in pregnancy and the role of paradoxical emboli, with a focus on PFO.

Congenital Heart Defects and Pulmonary Hypertension: The Heath–Edwards Paradigm

Pulmonary hypertension (PH) is a haemodynamic and pathophysiological disorder associated with a variety of cardiovascular and respiratory conditions, including congenital heart disease (CHD). In this Chapter, we discuss the definition and pathophysiology of pulmonary arterial hypertension (PAH) related to CHD (PAH-CHD) and its evolution, from Victor Eisenmenger’s first report, to its systematic description by Paul Wood. We focus not only on lung histopathology, but also describe current knowledge on cardiac adaptation to PH and the effect of PAH on the large pulmonary arteries. Finally, we introduce the concept of operability of cardiac defects in the presence of PAH and describe current recommendations for identifying PAH-CHD patients who may benefit from repair of their defect, as opposed to those in whom the defect should not be closed.

P7 The use of novel oral anticoagulants in patients with adult congenital heart disease

Introduction Novel oral anticoagulants (NOACs) are increasingly used in patients with arrhythmias to prevent embolic events. Simplicity of use, with no requirement for INR monitoring and dose alteration, has driven the rise in NOAC prescription for patients with adult congenital heart disease (ACHD). We provide a summary of one centre’s experience of the use of NOACs in ACHD. Methods All patients with ACHD treated with NOACs in our heart centre were retrospectively enrolled and data was collected from clinic letters. Results Between November 2012 and February 2015, 13 patients with ACHD in the Oxford Heart Centre were prescribed NOACs (dabigatran: n = 7; rivaroxaban: n = 5; apixaban: n = 1) for atrial flutter (n = 5), atrial fibrillation (n = 3), supraventricular tachycardia, Fontan circulation, previous pulmonary emboli and a blind ending pulmonary artery. The use of NOACs was acceptable in ACHD patients with learning difficulties, poor venous access, and for whom travel to hospital is difficult. Overall four patients reported side-effects after starting NOACs. Two of those on dabigatran experienced gastrointestinal symptoms. One patient on rivaroxaban reported bleeding from a Hickman line and menorrhagia that was improved by the progestogen-only pill. Another patient on rivaroxaban reported migraines. Conclusions Warfarin remains first line for arrhythmias in ACHD, however, the use of NOACs in such patients is increasing. The lack of regular INR measurements and dose alterations is particularly convenient in this population, who have undergone multiple medical interventions in their lifetime, and side effects are infrequent.