Jane Somerville

Repaired coarctation: a “cost-effective” approach to identify complications in adults

OBJECTIVESnThe study was done to determine the most cost-effective approach to follow adults after repair of coarctation of the aorta.nnnBACKGROUNDnRecoarctation and/or aneurysm formation following surgical repair or angioplasty for coarctation of the aorta carry a significant morbidity and mortality. Various screening tests to detect such complications are used, but little is known of their sensitivities and specificities; as a consequence, the most cost-effective approach to follow such patients is undefined.nnnMETHODSnRetrospective analysis was done on the sensitivity and specificity of symptomatology, physical examination, electrocardiogram, chest radiograph, exercise testing and transthoracic echocardiography to detect recoarctation and/or aneurysm formation in 84 adult patients following surgical repair or angioplasty of coarctation of the aorta, using magnetic resonance imaging (MRI) as the gold standard test.nnnRESULTSnEchocardiography had the highest sensitivity in detecting recoarctation (87%) and chest radiograph the highest sensitivity in detecting aneurysm formation (67%). Combined clinical visit and echocardiography had a high sensitivity for diagnosing recoarctation and/or aneurysm formation (97%), but performing a clinical visit and an MRI on every patient without any prior screening test emerged as the most cost-effective strategy.nnnCONCLUSIONSnThe most cost-effective approach to diagnose complications at the site of repair in patients after surgical repair or balloon angioplasty of coarctation of the aorta appears to be the combination of clinical assessment and MRI scan on every patient. If MRI resources are scant, performing a clinical assessment plus a transthoracic echocardiography and an MRI on patients with positive results is an acceptable alternative.

Amiodarone-Associated Thyroid Dysfunction Risk Factors in Adults With Congenital Heart Disease

BACKGROUNDnAmiodarone is widely used in adults with congenital heart disease, but no systematic study has been published on its effects on thyroid function in these patients. A retrospective study was performed to examine the frequency of amiodarone-associated thyroid dysfunction in adults with congenital heart disease and to identify any contributing factors.nnnMETHODS AND RESULTSnAll adults (16 to 60 years old) with congenital heart disease were identified from a database if they had no preexisting thyroid disease, had taken amiodarone for >/=6 months, and were currently followed up by 1 consultant (J.S.). Ninety-two patients were found and evaluated for thyroid status and cardiac complications. A case-control analysis was performed, with patients matched for duration of amiodarone therapy. Of the 92 patients (age, 34.9+/-10.2 years; range, 18 to 60 years), 36% developed thyroid dysfunction: 19 became hyperthyroid and 14 hypothyroid. Female sex and complex cyanotic heart disease were significant risk factors for developing thyroid dysfunction (odds ratios, 3.0 and 7.00; P=0.04 and 0.01, respectively). Previous Fontan-type surgery also appeared to be a risk factor for developing thyrotoxicosis (odds ratio, 4.0; P=0.17), and amiodarone >200 mg/d a risk factor for thyroid dysfunction (odds ratio, 4.0; P=0.60).nnnCONCLUSIONSnAmiodarone-associated thyroid dysfunction is common in adults with congenital heart disease. Women and those with complex cyanotic lesions are at particular risk, as patients may be who have had Fontan-type surgery or are taking >200 mg/d of amiodarone. Amiodarone should be used only when other antiarrhythmics are ineffective or contraindicated. Vigilance is required to detect and treat thyroid dysfunction.

Outcome of pregnancy in patients with congenitally corrected transposition of the great arteries.

To assess maternal and fetal outcome of pregnancy in patients with congenitally corrected transposition of the great arteries, we reviewed 19 patients (18 retrospectively) who had 45 pregnancies. Their ages ranged from 18 to 40 years (mean 27) at the time of pregnancy. Thirty-six percent of the pregnancies were undertaken while patients were cyanosed, 7% in patients with unpaced complete heart block, and 16% were undertaken after surgical repair of the associated anomalies. Change in functional class and maternal cardiovascular complications during pregnancy were analyzed as well as number of live births, miscarriages, elective termination of pregnancies, timing of delivery, and incidence of cardiac defects in the live offspring. Five patients (26% of patients) developed cardiovascular complications during pregnancy, namely congestive heart failure (3 patients), worsening cyanosis (1 patient), and cerebrovascular accident (1 patient). No maternal deaths occurred. There were 27 live births (60%), 12 miscarriages (27%), and 6 elective terminations of pregnancy (13%). Cyanosis was a significant risk factor for miscarriage. One live offspring had congenital heart disease. Close follow-up of these patients during pregnancy, by a team of experienced physicians, is recommended.

Atrial flutter in grown-up congenital heart (GUCH) patients. Clinical characteristics of affected population.

OBJECTIVEnTo define occurrence, lesions and clinical characteristics of grown-up congenital heart (GUCH) patients who develop atrial flutter (AFL).nnnDESIGNnAll GUCH patients who presented as inpatients or outpatients with documented sustained AFL between 1996 and 1998 were studied prospectively. Retrospective review of case notes for basic data relating to underlying anomaly, prior surgery and age at onset of AFL, enquiry into events before the first attack. Clinical state was assessed by Ability Index before AFL and at last visit.nnnSETTINGnDesignated quaternary service for GUC in a tertiary referral centre.nnnRESULTSnFrom October 1996 to April 1998, 100 consecutive patients (49 female) aged 17-77 (mean 35) years, who presented to the GUCH Unit at Royal Brompton Hospital with a sustained attack of AEL documented by a 12 lead electrocardiogram were studied. Four basic cardiac anomalies accounted for 75% patients: one ventricle (26), atrial septal defect (ASD) (19), transposition of great arteries (TGA) (17) and Tetralogy of Fallot (13). AFL occurred occasionally in small ventricular septal defect (VSD), congenital corrected TGA (CC-TGA), pulmonary stenosis and pulmonary atresia with or without VSD. 86/100 patients had undergone cardiac surgery: Fontan 19 (22%), reconstruction of right ventricular outflow tract 17 (20%), closure of ASD 15 (17%), Mustard for TGA 13 (15%), and other palliative surgery 22 (26%). AFL occurred in natural history (unoperated) in 14 (14%) mostly in CC-TGA, ASD and Fallot. Age at first attack was 6-64 (mean 28) years with the first attack occurring at younger age after Mustard (22+/-7 years) and Fontan (24+/-7), than in un-operated ASD (46+/-13) and CCTGA (31+/-10). Haemodynamic abnormalities from anatomical causes were present in 62/74 (84%) patients who had undergone reparative surgery and included venous pathway obstruction, pulmonary regurgitation and pulmonary hypertension. Additional factors which could have precipitated AFL in prone patients were present in 63. New symptoms appeared in 96 patients with the first attack of AFL. Ability Index prior to onset in 90 patients who have been followed-up for more than 1 year since the first onset was 1 in 52, 2 in 31, 3 in 6 and 4 in 1 patients. At the last visit (mean time from the first onset 6.6+/-4.7 years), only 9 patients remained with Ability Index 1, 43 in 2, 20 in 3 and 18 in 4 despite return to sinus rhythm.nnnCONCLUSIONnOne ventricle heart, ASD, transposition of great arteries and Tetrology of Fallot are the most common underlying anomalies in GUCH patients who develop AFL. It is less commonly seen in unoperated patients. When occurs AFL compromises patients activities and deteriorates the clinical condition. Residual or developed haemodynamic abnormalities and precipitating factor are often present in this patients, hence full investigation and close follow up are necessary once AFL develops.

Effect of vanadium on serum cholesterol.

Abstract Twelve patients were treated with oral vanadium for 6 months. Nine patients had hypercholesterolemia, and 7 of these had ischemic heart disease. Three patients had ischemic heart disease and normal serum cholesterol. No change in serum cholesterol or lipoprotein patterns was found. The clinical course of the patients remained as would be expected for the natural history of the disease, and there was no alteration in the xanthomata. Toxic side effects occurred in 6 patients. In this limited study, therefore, we have found no evidence that diammonium vanadotartrate lowers serum cholesterol.

Effects of Chronic Hypoxemia on Chemosensitivity in Patients With Univentricular Heart

OBJECTIVESnWe sought to compare the arterial blood gas chemosensitivity in relation to exercise ventilatory response in patients with univentricular heart and cyanosis and in patients with univentricular heart and Fontan-type circulation without cyanosis.nnnBACKGROUNDnPatients with univentricular heart demonstrate excessive ventilation during exercise. Chronic hypoxemia may alter chemoreceptor function, affecting ventilation.nnnMETHODSnCardiopulmonary exercise testing was performed in 10 patients with rest or stress-induced cyanosis (cyanotic group: mean age +/- SE 30.5 +/- 2.3 years; 5 men), 8 patients without cyanosis with Fontan-type circulation (Fontan group: mean age 29.4 +/- 1.5 years; 4 men) and 10 healthy control subjects (normal group: mean age 30.7 +/- 1.9 years; 5 men). Hypoxic and hypercapnic chemosensitivity were assessed by using transient inhalations of pure nitrogen and the rebreathing of 7% CO2 in 93% O2, respectively.nnnRESULTSnPeak O2 consumption was comparable in both patient groups (21.7 +/- 2.5 [cyanotic group] vs. 21.0 +/- 1.9 ml/kg per min [Fontan group]) but was lower than that in the normal group (34.7 +/- 1.9 ml/kg per min). The ventilatory response to exercise, characterized by the regression slope relating minute ventilation to CO2 output, was higher in the cyanotic group (43.4 +/- 4.0) than in the Fontan group (31.4 +/- 3.0, p = 0.02) and the normal group (23.1 +/- 1.1). Hypoxic chemosensitivity was blunted in the cyanotic group compared with that in the Fontan and normal groups (0.148 vs. 0.448 [p = 0.02] vs. 0.311 liter/min per percent arterial O2 saturation, respectively) and did not correlate with the ventilatory response to exercise (r = -0.36, p = 0.29). In contrast, hypercapnic chemosensitivity represented by the slope of the hypercapnic-ventilatory response line was similar in the cyanotic, Fontan and normal groups (1.71 vs. 1.76 vs. 1.70 liter/min per mm Hg, respectively), but the response line had shifted to the left in the cyanotic group (x intercept = 31.9 vs. 39.9 mm Hg [p = 0.026]), compared with 45.2 mm Hg in normal subjects. These findings suggest that in the cyanotic group, ventilation is greater for a given level of arterial CO2 tension and thus may partly explain the increased exercise ventilatory response in this group.nnnCONCLUSIONSnHypoxic chemosensitivity is blunted in patients with univentricular heart and cyanosis and does not determine the exercise ventilatory response. CO2 elimination appears more important. The blunting of hypoxic chemosensitivity is reversible once chronic hypoxemia is relieved, as evident in the Fontan group.

Sudden death in an adult with a small ventricular septal defect and an aneurysmal membranous septum

An apparently healthy man of 26 years of age suddenly died. He was known to have had a small ventricular septal defect and complete right bundle branch block from early childhood. At post-mortem examination the small ventricular septal defect was found associated with an aneurysm of the membranous septum. Histological examination showed a normal atrioventricular node and bundle, adjacent to the aneurysm. There was fibrous interruption at the commencement of the right bundle branch, which was considered the basis of the bundle branch block. It was also considered that the right bundle branch block was of the hereditary type and that this, rather than the aneurysm of the membranous septum, was responsible for the unexpected death.

QRS width in right bundle branch block. Accuracy and reproducibility of manual measurement

BACKGROUNDnThe QRS prolongation and its relation to malignant ventricular arrhythmias are topics of interest. Controversies exist about the methodology of measuring the QRS. The aim of this study was to assess the accuracy and reproducibility of manual measurement of the QRS in standard electrocardiograms in patients with right bundle branch block and compare results with computer reading.nnnMETHODS AND RESULTSnFive experienced cardiologists at different levels of training were required to measure QRS duration in 30 electrocardiograms with different degrees of right bundle branch block collected from 24 randomly selected patients who had had radical repairs of tetralogy of Fallot. In each set of electrocardiograms there were six records which had been duplicated. The observers were neither told the purpose of the study nor how the electrocardiograms had been obtained, nor informed that some of the electrocardiograms were duplicates. Photocopies were identified by number, covering the patients name and computerised measurement. Significant differences were found in the measurement of QRS in the same ECG calculated twice by the same observer (with an absolute variation up to 50 ms), within different observers (P=0.037) and measured manually or by computer (P=0.019). The width of the QRS did not influence the measurements as the biggest intra-observer variation (50 ms) was observed for relatively wide complex (median value between the two measurements 155 ms) and the biggest inter-observer (60 ms) for narrow complex (median value between the five measurements 110 ms). The QRS morphology appeared to influence the measurements, as the intra- and inter-observer variations were more consistent in the presence of obvious notching, slurrings and terminal slow vectors.nnnCONCLUSIONSnMeasurement of QRS is difficult, can be operator dependent and influenced by the presence of conduction abnormalities which reduce its accuracy and reproducibility.

Complex pulmonary atresia in an adult: natural history, unusual pathology and mode of death

A patient with unrepaired complex pulmonary atresia had a normal life, achieving two successful pregnancies, until the age of 44 years. Confluent central pulmonary arteries were supplied by a fistuious communication from the left coronary artery, and from other collateral arteries arising from the underside of the aortic arch. Unusual aneurysms were present. Death at the age of 46 resulted from dissection and rupture of an aneurysmal dilation of the pulmonary trunk.

A postmortem review of congenital cardiac malformations in a series of 180 adults, over the age of 16 years, born between 1865 and 1980.

The evolution of diagnosis and treatment of congenital heart malformations can be traced through patients surviving into adulthood. We reviewed the heart specimens from 180 patients aged 16 to 86 years and considered the morphological features, the nature of any interventional procedures, and the events leading to death. Based on the mode of clinical presentation, 33 cases were considered covert, and the remaining 147 cases were known or suspected to have a cardiac abnormality during life. Of the symptomatic cases, 60 had no surgical intervention, whereas 167 surgical procedures had been performed in the remaining 87 cases. Acquired heart disease was noted in 7 of the covert cases and in 16 of the symptomatic cases. Overall, there were only 3 instances of errors in clinical identification of significant morphological abnormalities, and 2 cases related to surgical procedures. This review emphasizes the value of autopsy examination for clinicopathologic correlations and the case for retention of cardiac specimens for teaching purposes.