John M. Simpson

Long-term efficacy of percutaneous transluminal coronary angioplasty (PTCA): Report from the national heart, lung, and blood institute PTCA registry

The NHLBI PTCA Registry has collected data from 3,079 patients who underwent PTCA at 105 centers from September 1977 through September 1981 that document the initial risks and benefits of PTCA. A subgroup of 2,272 patients at 65 centers was chosen to examine the long-term effects of PTCA (97% follow-up). All patients were followed for 1 year, 191 for 3 years and 57 for 4 years. Initial success occurred in 1,397 (61%), and 72% remained improved at 1 year with no further procedures; during the first year of follow-up, 14% had repeat PTCA, 12% had CABG, 3% had MI and 1.6% died. After 1 year, 67% were asymptomatic; of these, 52% had no other procedure, 7% had a second PTCA and 8% had CABG. Follow-up at 2 to 4 years was similar except that there were few repeat PTCA or CABG procedures after 1 year. The annual mortality rate after PTCA in patients with 1-vessel diseases was less than 1% per year and with multivessel CAD, 3% per year. Thus, successful PTCA alone results in sustained improvement in 84% of patients; 59% were asymptomatic (12% had repeat PTCA). PTCA offers extended effective therapy in selected patients with CAD.

Targeted Neonatal Echocardiography in the Neonatal Intensive Care Unit: Practice Guidelines and Recommendations for Training Writing group of the American Society of Echocardiography (ASE) in collaboration with the European Association of Echocardiography (EAE) and the Association for European Pediatric Cardiologists (AEPC)

Luc Mertens, MD, PhD, FASE, FESC, Istvan Seri, MD, PhD, HonD, Jan Marek, MD, PhD, FESC, Romaine Arlettaz, MD, Piers Barker, MD, FASE, Patrick McNamara, MD, MB, FRCPC, Anita J. Moon-Grady, MD, Patrick D. Coon, RDCS, FASE, Shahab Noori, MD, RDCS, John Simpson, MD, FRCP, FESC, Wyman W. Lai, MD, MPH, FASE, Toronto, Ontario, Canada; Los Angeles and San Francisco, California; London, United Kingdom; Zurich, Switzerland; Durham, North Carolina; Philadelphia, Pennsylvania; New York, New York

Patterns of recurrence of congenital heart disease: an analysis of 6,640 consecutive pregnancies evaluated by detailed fetal echocardiography.

OBJECTIVES We sought to investigate the pattern of recurrence of congenital heart disease (CHD) where there is one or more affected first-degree relative. BACKGROUND There are little data on patterns of recurrence of different types of CHD. Analysis of a fetal series allows a high ascertainment of affected cases. METHODS We performed an analysis of referrals for detailed fetal echocardiography to a tertiary fetal cardiology unit, where there was a first-degree family history of CHD from 1990 to the end of 1999. Data were entered prospectively on a computerized database. Recurrences were exactly concordant if CHD was identical to the index case, and concordant for the group if belonging to a similar group of CHD. RESULTS A recurrence of CHD was seen in 178 (2.7%) of 6,640 pregnancies. The referral numbers for sibling, maternal, or paternal CHD cases were 5,151, 1,119, and 370, respectively. Exact concordance was seen in 37% of cases (range 0% to 80%), and group concordance was seen in 44%. In families where there were two or more recurrences, the exact concordance rate was 55%. Exact concordance rates were particularly high for isolated atrioventricular septal defects (4 of 5 [80%]) and laterality defects (7 of 11 [64%]). CONCLUSIONS The concordance rates of different types of CHD vary widely. Accurate diagnosis of the index case is essential for reliable counseling on patterns of recurrence. Minor CHD in the index case does not exclude more severe disease in recurrences. There appears to be significant under-referral for fetal echocardiography in paternal CHD.

Percutaneous angioplasty of stenoses of bypass grafts or of bypass graft anastomotic sites.

Forty patients who had stenoses of coronary artery bypass grafts or stenoses of the proximal or distal coronary insertion sites were treated with percutaneous transluminal angioplasty (PTA). PTA was successful in 78% of the patients. Mean stenoses were diminished from 86% to 34% in luminal diameter. One patient needed emergency coronary artery bypass grafting. There were no deaths associated with PTA. Follow-up angiography showed continued patency in asymptomatic patients, whereas in all but 1 patient with recurrent symptoms restenosis had developed at the PTA site. In 38% of patients restenosis developed after successful PTA; the highest restenosis rate was after PTA of aorta/graft anastomoses or proximal graft stenoses. In conclusion, PTA is a successful, low risk alternative to repeat coronary artery bypass grafting, although the restenosis rates at some PTA sites are high.

Targeted Neonatal Echocardiography in the Neonatal Intensive Care Unit: Practice Guidelines and Recommendations for Training

AAP : American Academy of Pediatrics AEPC : Association for European Paediatric Cardiology ASE : American Society of Echocardiography CDH : Congenital diaphragmatic hernia CHD : Congenital heart disease EAE : European Association of Echocardiography ECMO : Extracorporeal membrane oxygenation EF : Ejection fraction LV : Left ventricular MPI : Myocardial performance index mVCFc : Mean velocity of circumferential fiber shortening NICU : Neonatal intensive care unit PA : Pulmonary artery PDA : Patent ductus arteriosus RA : Right atrial RV : Right ventricular RVSp : Right ventricular systolic pressure SF : Shortening fraction SVC : Superior vena cava TEE : Transesophageal echocardiography TNE : Targeted neonatal echocardiography TVI : Time-velocity integral 2D : Two-dimensional VLBW : Very low birth weight The role of echocardiography in the neonatal intensive care unit (NICU) has changed over the past few years. Previously, nearly all echocardiographic studies in the NICU were performed by pediatric cardiologists to diagnose or monitor congenital heart disease (CHD) and to screen for patent ductus arteriosus (PDA). More recently, neonatologists have become interested in the echocardiographic assessment of hemodynamic instability in infants. The terms functional echocardiography and point-of-care echocardiography have been introduced to describe the use of echocardiography as an adjunct in the clinical assessment of the hemodynamic status in neonates.1–4 The increasing availability of echocardiography, with miniaturization of the technology, has resulted in more widespread use of echocardiography in NICUs around the world.5 Perhaps the most significant challenge for the application of so-called functional studies is that newborns in the NICU with hemodynamic instability are at a much higher risk for having underlying CHD. In addition, newborns in the NICU are unique in that they are in the process of …

Review of diagnosis, treatment, and outcome of fetal atrial flutter compared with supraventricular tachycardia.

Objective: To review the diagnosis, treatment, and outcome of fetal atrial flutter compared with supraventricular tachycardia. Design: Retrospective review of published reports: 11 papers about fetal tachyarrhythmia published between 1991 and 2002 were selected for review. Main outcome measures: All selected studies were analysed for the type of arrhythmia, degree of atrioventricular block in atrial flutter, occurrence of hydrops fetalis, gestational age at diagnosis, first and second line drug treatment, associated cardiac and extracardiac malformations, and mortality of the fetuses. Results: Atrial flutter accounted for 26.2% of all cases of fetal tachyarrhythmias, and supraventricular tachycardia for 73.2%. Hydrops fetalis was reported in 38.6% and 40.5% of fetuses with atrial flutter and supraventricular tachycardia, respectively (NS). Hydropic fetuses with atrial flutter had higher ventricular rates (median 240 beats/min, range 240–300) than non-hydropic fetuses (220 beats/min, range 200–310) (p = 0.02), whereas the atrial rates were not significantly different (median 450 beats/min, range 370–500). Digoxin treatment resulted in a higher conversion rate in non-hydropic fetuses with fetal tachyarrhythmias than in hydropic fetuses (p < 0.001). The overall mortality of atrial flutter was similar to that of supraventricular tachycardia, at 8.0% v 8.9% (p = 0.7). Conclusions: The prevalence of hydrops fetalis did not differ in fetal atrial flutter and supraventricular tachycardia with 1:1 conduction. There was no difference between the response rate to digoxin in fetus with atrial flutter or supraventricular tachycardia. Mortality was similar in the two types of tachyarrhythmia.

Timing of presentation and postnatal outcome of infants suspected of having coarctation of the aorta during fetal life

Objective: To report the timing of presentation and clinical profile of a cohort of fetuses with normal main cardiac connections but fetal echocardiographic signs suggestive of coarctation of the aorta. Design: Retrospective observational study. Setting: Tertiary fetal and paediatric cardiology centre. Patients: Between 1 January 1998 and 31 December 2002, 174 fetuses were studied, of whom 144 infants were born alive. Main outcome measures: Of the 144 liveborn infants, 43 had coarctation of the aorta, four had interruption of the aortic arch, and one was managed as having hypoplastic left heart syndrome. Hemianomalous pulmonary venous drainage was diagnosed in two infants. Three infants with coarctation presented late at 7–13 weeks of age, 6–12 weeks after closure of the arterial duct. Fetuses with cardiac asymmetry had a higher incidence of left superior vena cava than a control group. For fetuses with cardiac asymmetry, the incidence of left superior vena cava and ventricular septal defects was similar in infants who proved to have coarctation postnatally and in those who did not. The 30 day and one year surgical mortality of infants having repair of coarctation of the aorta was two of 41 (4.9%, 95% confidence interval (CI) 0.6 to 16.0). All cause mortality of liveborn infants with any abnormality of the aortic arch was five of 48 (10.4%, 95% CI 3.5 to 22.7) at 30 days and one year, which was heavily influenced by prematurity and extracardiac abnormalities. Conclusions: Precise diagnosis of coarctation of the aorta during fetal life remains difficult. Coarctation of the aorta may present several weeks after closure of the arterial duct and sequential echocardiography is recommended.

Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis

BACKGROUND AND AIMS Staged reconstructive surgery has radically altered the prognosis of hypoplastic left heart syndrome (HLHS). Antenatal diagnosis allows for appropriate counselling, and time to consider treatment options. We report outcome from a centre where most cases are antenatally diagnosed and delivered on site. METHODS Information was collated on 188 consecutive cases of HLHS between 1995 and 2000, including timing of diagnosis, outcome of pregnancy, and age and outcome at each stage of surgery. At Guys Hospital, 174 cases were diagnosed antenatally, of whom 50 underwent surgery. Fourteen others (five diagnosed antenatally at other centres, and nine diagnosed postnatally) also underwent surgery. RESULTS Survival after stage I (the Norwood operation) was 52% (33/64). Postoperative survival after stage II (the hemi-Fontan operation, performed in 29), and stage III (the Fontan operation, performed in 10), was 100%. Two late deaths occurred 3 and 10 months after stage II, giving overall survival of 48% (31/64). At follow up, three children have neurological impairment, and one had poor right ventricular function necessitating cardiac transplantation. CONCLUSIONS Antenatal diagnosis allows informed decisions about treatment options, and facilitates preoperative care. Mortality following stage I is high, irrespective of timing of diagnosis, but medium term outcome for survivors is good.

Cardiac Magnetic Resonance Imaging After Stage I Norwood Operation for Hypoplastic Left Heart Syndrome

Background— After the Norwood operation, a patient’s suitability for proceeding to a bidirectional cavopulmonary connection (BCPC) is assessed by a combination of echocardiography and diagnostic cardiac catheterization. In this study, we describe the results of 37 patients who underwent cardiovascular magnetic resonance (MR) assessment before BCPC. Methods and Results— Cardiovascular MR and echocardiography were performed in 37 infants with hypoplastic left heart syndrome before BCPC, and the findings were compared with surgical findings. MR assessment of ventricular function and valvar regurgitation were compared with echocardiography. MR exhibited high sensitivity and specificity for identification of neoaortic (sensitivity 86%, specificity 97%) and left pulmonary artery (sensitivity 100%, specificity 94%) obstruction. Echocardiography exhibited poor sensitivity for identification of vascular stenosis. The mean right ventricular ejection fraction calculated from the MR data was 50±10%. There was general agreement between MR and echocardiographic measures of ventricular function, although patients with good function on echocardiography demonstrated a wide range of ejection fractions. There was good agreement between MR and echocardiography for identification of valvar regurgitation. Conclusions— Cardiovascular MR can be used to define ventricular and valvar function and vascular anatomy in infants with hypoplastic left heart syndrome after the Norwood operation. We have shown how this information can be used to plan the BCPC and identify any revisions or additional valvar surgery.

Genotypic and Phenotypic Characterization of Drug-Resistant Mycobacterium tuberculosis Isolates from Rural Districts of the Western Cape Province of South Africa

ABSTRACT Genotypic and phenotypic analysis of drug-resistant Mycobacterium tuberculosis isolates from the Western Cape Province of South Africa showed that drug resistance is widespread and recently transmitted. Multidrug-resistant (MDR) isolates comprise 40% of this collection, and a large pool of isoniazid monoresistance may be a future source of MDR tuberculosis.