The history of Helen Taussig is well known. Contrary to popular belief, however, she was not the first director of the pediatric cardiology clinic at the Harriet Lane Home for Invalid Children. She was second director, succeeding Clifton B. Leech. Edwards A. Park, Chief of Pediatrics at Johns Hopkins, appointed Leech the first director of the pediatric cardiac clinic in the fall of 1928. In this vignette, I summarize the contributions of Clifton Leech to the development of paediatric cardiology.

Before paediatric cardiology emerged as a specialty in the mid 20th century, a body of literature had developed over centuries devoted to description of congenitally malformed hearts. In this review, we have selected highlights from such texts written during the period of 100 years from 1814 to 1914, demonstrating their potential relevance to controversies occurring during the twentieth century in the categorisation of such hearts. We begin in 1814, with the first wide-ranging book devoted to congenital cardiac malformations. We end with a publication from 1914, because it included an illustration of the first electrocardiogram in a text devoted to paediatric disease. As we will show, these works from the 19th and early 20th centuries reflect topics still relevant today, namely the aetiology of cardiac malformations, clinicopathologic correlations, attempts at classification, and lack of effective treatments. Attention to their content could have served to ameliorate controversies, some of them ongoing.

Rheumatic heart disease is the only residual morbidity, and the sole cause of mortality, from rheumatic fever. Echocardiography is ideally suited to confirm and follow the course of rheumatic heart disease. Additionally, both minimal valvar pathology in children, and extensive valvar pathology in adults, may not cause a murmur and can be detected only by echocardiography. Whenever possible, echocardiography should be routinely employed for management of patients with rheumatic fever or suspected rheumatic fever.

Mixed venous saturation of oxygen has for some time been used as a tool to assess the adequacy of systemic delivery of oxygen in intensive care. In the post-operative child with cardiac disease, it is increasingly being used to assess adequacy of cardiac output. In many of these patients, true sampling of mixed venous saturation of oxygen is not possible due to the infrequent use of pulmonary arterial catheters, or the presence of intra-cardiac left to right shunting leading to mixing of systemic and pulmonary venous blood. The use of saturation of oxygen in the central venous blood as a surrogate for mixed venous saturation of oxygen has been widely investigated in adults, but its use remains controversial. In this review, we discuss the theory behind the use of mixed venous saturation of oxygen in evaluating cardiac output, the problems pertinent to those patients with congenitally malformed hearts, and explore the evidence for central venous saturation of oxygen as a surrogate for mixed venous saturation of oxygen, and its use as a therapeutic target to improve outcomes in this population of patients.

Catecholaminergic polymorphic ventricular tachycardia is a rare entity that can occur in children without cardiac disease and with a normal QT interval. It may cause syncope, convulsions, and sudden death during physical activity or emotional distress. We report the clinical features, treatment, and follow-up of 16 children with this diagnosis, emphasizing the potentially fatal nature of the disease.

The mean age of patients at the onset of symptoms and at the time of diagnosis was 7.8 plus or minus 2.5 years, and 10.6 plus or minus 3.5 years, respectively. Syncope was the main complaint in 11, and 7 were treated as erroneously as having epilepsy. Diagnosis was confirmed by exercise and/or infusion of isoproterenol. Once the diagnosis was made, we started propranolol in all patients, and added verapamil if ventricular tachycardia was still inducible on a treadmill exercise test. An intracardiac defibrillator was implanted in 4 patients. Of the 16 patients, 4 died suddenly, giving a rate of mortality of 25%. In 2 of those dying suddenly, there was evidence of poor compliance to the recommended treatment. Another 2 patients had been resusciated because of sudden cardiac arrest.

Catecholaminergic polymorphic ventricular tachycardia must be considered in the differential diagnosis of syncope in children without heart disease but with a normal QT interval. Medical treatment with propranolol and verapamil may decrease the incidence of arrhythmia. Implantation of intracardiac defibrillators should be considered in those resistant to drug therapy. Delay in diagnosis, and inadequate treatment, can result in sudden cardiac death.

Echocardiographic and radionuclide techniques are the most common techniques currently used to assess ventricular function. The aim of our study was to assess the performance of two well-known methods for analysis of gated myocardial perfusion with comparison to echocardiography in children. We analyzed the data from 64 children referred for myocardial perfusion scintigraphy. All underwent echocardiography and stress gated myocardial perfusion scintigraphy, except 5 who had only rest scintigraphy. We calculated the left ventricular volumes and ejection fractions. The parametric information from the echocardiographic reports was compared to the data obtained using gated myocardial perfusion. The reliability between the quantitative gated scintigraphy, the Emory cardiac toolbox, and echocardiography for end-diastolic and end-systolic volumes and ejection fractions in studies performed at rest were calculated at 0.85, 0.86 and 0.97, respectively using Cronbach’s alpha coefficient. The values in stress studies were 0.83, 0.86 and 0.90, respectively. There was a high correlation, with r more than 0.88, between quantitative gated scintigraphy and the Emory cardiac toolbox for ejection fractions, and end-diastolic and end-systolic volumes, in studies performed both under stress and at rest. Weak correlation was observed between quantitative gated scintigraphy and the Emory cardiac toolbox compared to echocardiography, especially when considering ventricular volumes in stress studies. Although high correlation was observed between quantitative gated scintigraphy and the Emory cardiac toolbox, comparison with echocardiography showed poor agreement for both, meaning that scintigraphy is less suitable for assessing left ventricular volumes, and less reliable in assessment of ejection fractions. Echocardiography seems to remain the most widely used and reliable technique for this part of the diagnostic work up.

Our aim was to determine whether the index of left ventricular mass increases during the acute and subacute phase of Kawasaki disease, and to investigate any relationship between this index and clinical and echocardiographic variables. We performed, therefore, a retrospective study of 66 children with Kawasaki disease, having a mean age of 2.85 years, comparing the findings with those obtained from 57 normal controls, having a mean age of 2.99 years. The data from the patients used for comparison was obtained during the acute and subacute phase of the illness, as well as during the phase of convalescence. We performed correlation analysis of the findings during the acute and subacute phase, determining the relationship between the index of left ventricular mass and other variables. The index was higher (p = 0.0461), and the velocity of propagation of left ventricular early diastolic flow was lower (p < 0.0001), during the acute and subacute phase when compared to control values. The index then reduced (p = 0.0001) during the phase of convalescent when compared to the acute and subacute phase. Levels of albumin in the serum (p = 0.0193), peak E velocity (p = 0.0479), and velocity of propagation (p = 0.0360) were found to be related to the index of left ventricular mass. Significant relationships were found between the differences in this index and differences in body weight when findings during the acute and subacute phase were compared to those of the phase of convalescence. The index of left ventricular mass, therefore, is increased during the acute and subacute phase of Kawasaki disease, and is associated with altered diastolic indexes. This elevation may be due to generalized myocardial swelling from acute inflammation and increased vascular permeability. Measuring this index as a potential predictor of diastolic function should be added to studies of cardiac function during the acute and subacute phase of Kawasaki disease.

The supramitral ring is a rare congenital malformation formed by presence of a ridge of connective tissue, usually attached at or above the mitral annulus. The incidence and clinical presentation is highly variable due to difficulty in diagnosis. A review of autopsied congenital heart diseases at our institute over a 17-year-period revealed 24 cases of supramitral ring. These were classified with respect to the morphology of the ridge and the presence of associated cardiac lesions. The ring was found in 1.5% of the autopsied specimens of congenitally malformed hearts, and in 37.5% of those with obstructed left-sided inflow tracts. The majority of the specimens came from children (79.2%). A clinical diagnosis had been made in only two. In one-third of the cases, the ring was associated with incomplete Shone’s complex. Varied anomalies were seen in others, chiefly ventricular septal defects. An interesting association was the presence of rheumatic mitral valvar disease, found in 3 cases. There was no difference in the completeness or width of the supramitral ridge in the hearts from those with or without Shone’s complex. Circumferential rings were fleshy and stenosing, while incomplete rings had variable locations and stenosis. The presence of a supramitral ring may be underestimated due to association with other cardiac anomalies, both congenital and acquired. Since the ridge need not always produce stenosis, the correct designation would be simply a supramitral ring.

Uncertainty exists regarding the degree to which infants with congenitally malformed hearts are at risk of behavioural disorders in childhood. Data was collected as part of a randomized clinical trial involving 155 children with surgically corrected transposition (concordant atrioventricular and ventriculo-arterial connections or alignments). As infants, they underwent the arterial switch operation, involving deep hypothermia with predominantly total circulatory arrest or predominantly low-flow continuous cardiopulmonary bypass as the method of providing support to the vital organs. Parents completed the Child Behavior Checklist when the patients were aged 4 and 8 years, and the Connors’ Parent Rating Scale at the age of 8 years. When the children were aged 8, teachers completed the Teacher’s Report Form and the Connors’ Teacher Rating Scale. In the cohort as a whole, the frequencies of behavioural problems identified by both parents and teachers were elevated, particularly on the scales for competence of the Child Behavior Checklist, and the Adaptive scales of the Teacher’s Report Form. Approximately 1 in 5 patients had scores for Total Problem Behavior in the range of clinical concern on both the Child Behavior Checklist and the Teacher’s Report Form. Few differences were found, however, according to the method of operative treatment. Postoperative seizures were associated with social and attention problems. Children experiencing academic problems at the age of 8 showed a larger increase in behavioural problems between the ages of 4 and 8 than did children making adequate academic progress. Children with congenitally malformed hearts who underwent reparative surgery in infancy using a strategy of severe haemodilution and alpha stat are at increased risk of behavioural problems in middle childhood.

We describe the progress of a girl aged 5 years, who suffered blunt trauma to the chest producing a ventricular septal defect of 1 centimetre in diameter. The shunt generated a mild dilation of the left-sided chambers, and exertional dyspnoea. Three months later, therapeutic catheterisation revealed important diminution in the extent of shunting. We decided, therefore, not to close the defect, and she has since remained asymptomatic.

We report an infant with transposition, with the circumflex artery arising from the right coronary artery, who was diagnosed with cystic fibrosis following the occurrence of meconium ileus in the postoperative period.