Selective angiocardiography in congenital heart disease

Angiocardiography has come to be a highly specialized procedure and there are definite indications for doing it. In specific cases it offers clear-cut advantages. Technical improvements permit a single injection of a small amount of contrast substance. Selective angiocardiography is advised where the particular detail of a certain region in the heart is desired.     

Necrotizing enterocolitis — a medical approach to treatment

Ten cases of necrotizing enterocolitis occurred among 5400 infants delivered consecutively during a two and one-half year period at the Halifax Infirmary. Since this disease involved such extensive areas of the intestinal wall, medical management was employed exclusively, even when intestinal perforation occurred. Nine of the ten infants survived.In this series there was a high incidence of prematurity, prolonged interval between rupture of the placental membranes and delivery, severe asphyxia neonatorum and subsequent hypotension and metabolic acidosis. Blood cultures grew Klebsiella aerobacter or E. coli in 7/10 infants. Only two of these organisms were sensitive to kanamycin and all were resistant to ampicillin. Administration of ampicillin to the mothers did not protect these infants against necrotizing enterocolitis. Edema of the gut wall appeared to be an important early sign. Stricture formation occurred in 4/10 cases, all in the terminal ileum. These infants were operated upon only after the acute stage of the disease had subsided.     

Inheritance of congenital heart disease

Congenital heart defects (CHD) are the most common developmental anomalies and are the leading noninfectious cause of mortality in newborn babies. It has been estimated that between four and ten live-born infants per 1000 have a cardiac malformation (0.4 to 1.0%), 40% of which are diagnosed in the first year of life. The European Registration of Congenital Anomalies (EUROCAT) reported a prevalence of 58.9/10,000 live births in the northern part of the Netherlands (0.6%). Hoffman estimated that the true prevalence of CHD may be as high as 53 per 1000 pregnancies (5.3%), including a 20% occurrence of heart defects in spontaneous abortion, a 10% occurrence in stillbirth, and a 1% occurrence in live birth.     

Sudden unexpected death in children with congenital heart disease.

Of 18,000 children with organic heart disease evaluated at The Hospital for Sick Children, Toronto between 1940 and 1971, 33 died suddenly and unexpectedly between 1 and 21 years of age. Nine had discrete obstruction of the left ventricular outflow tract and five had muscular narrowing of the left ventricular outflow tract and five had muscular narrowing of the left ventricular outflow tract. Pulmonary vascular disease caused seven sudden deaths, and arrhythmias (usually due to atrioventricular block) caused seven more. Of the five other children who died suddenly three had transposition of the great arteries, one had a complex cyanotic heart defect and one had an anomalous course of the left coronary artery, which originated from the right sinus of Valsalva. With earlier investigation of aortic stenosis, earlier closure of ventricular septal defect to avoid pulmonary vascular disease, better design of artificial pacemakers and better investigation of patients with angina, many of these deaths will be avoided in the future.     

Myocardial perfusion reserve in adults with cyanotic congenital heart disease

In patients with cyanotic congenital heart disease (CCHD), a right-to-left shunt results in systemic hypoxemia. Systemic hypoxemia incites a compensatory erythrocytosis, which increases whole blood viscosity. We considered that these changes might adversely influence myocardial perfusion in CCHD patients. Basal and hyperemic (intravenous dipyridamole) perfusion measurements were obtained with [13N]ammonia positron emission tomographic imaging in left (LV) and right (RV) ventricular and septal myocardium in 14 adults with CCHD [age: 34.1 yr (SD 6.5)]; hematocrit: 62.2% (SD 4.8)] and 10 healthy controls [age: 34.1 yr (SD 6.5)]. In patients, basal perfusion measurements were higher in LV [0.77 (SD 0.24) vs. 0.55 ml x min(-1) x g(-1) (SD 0.09), P < 0.02], septum [0.71 (SD 0.16) vs. 0.49 ml x min(-1) x g(-1) (SD 0.09), P < 0.001], and RV [0.77 (SD 0.30) vs. 0.38 ml x min(-1) x g(-1) (SD 0.09), P < 0.001]. However, basal measurements normalized for the rate-pressure product were similar to those of controls. Calculated oxygen delivery relative to rate-pressure product was higher in the patients [2.2 (SD 0.8) vs. 1.6 (SD 0.4) x 10(-5) ml O2 x min(-1) x g tissue(-1) x (beats x mmHg)(-1) in the LV, P < 0.05, and 2.0 (SD 0.7) vs. 1.4 (SD 0.3) x 10(-5) ml O2 x min(-1) x g tissue(-1) x (beats x mmHg)(-1) in the septum, P < 0.01]. Hyperemic perfusion measurements in CCHD patients did not differ from controls [LV, 1.67 (SD 0.60) vs. 1.95 ml x min(-1) x g(-1) (SD 0.46); septum, 1.44 (SD 0.56) vs. 1.98 ml x min(-1) x g(-1) (SD 0.69); RV, 1.56 (SD 0.56) vs. 1.65 ml x min(-1) x g(-1) (SD 0.64), P = not significant], and coronary vascular resistances were comparable [LV, 55 (SD 25) vs. 48 mmHg x ml(-1) x g x min (SD 16); septum, 67 (SD 35) vs. 50 mmHg x ml(-1) x g x min (SD 21); RV, 59 (SD 26) vs. 61 mmHg x ml(-1) x g x min (SD 27), P = not significant]. These findings suggest that adult CCHD patients have remodeling of the coronary circulation to compensate for the rheologic changes attending chronic hypoxemia.     

New developments in adult congenital heart disease

Congenital heart disease (CHD) affects 0.8% of live births and over the past decades technical improvements and large-scale repair has led to increased survival into adulthood of over 95% of the new-born. A new group of patients, those who survived their congenital heart defect, has emerged but late complications including heart failure, pulmonary hypertension (PH), arrhythmias, aneurysms and endocarditis appeared numerous, with a huge impact on mortality and morbidity. However, innovations over the past years have changed the landscape of adult CHD dramatically. In the diagnostic process important improvements have been made in the use of MRI, biomarkers, e‑health concepts and 3D visualisation of anatomy. Care is now concentrated in specialised centres, with a continuous emphasis on education and the introduction of weekly multidisciplinary consultations on diagnosis and intervention. Surgery and percutaneous intervention have been refined and new concepts applied, further reducing the burden of the congenital malformations. Research has matured from case series to global networks. Currently, adults with CHD are still facing high risks of early mortality and morbidity. By global collaboration and continuous education and development and innovation of our diagnostic and therapeutic arsenal, we will improve the perspectives of these young patients.

     

Clues in diagnosing congenital heart disease.

A number of practical office and bedside clues to cardiac disease in infants and children have been passed on through the years. They relate to the history, to the inspection and palpation components of the physical examination, and to knowledge of the specific cardiac defects that are likely to be associated with certain clinical syndromes. With the possible exception of coarctation of the aorta, the clues are not diagnostically specific. In many instances, however, they serve to narrow a broad array of diagnostic possibilities to 2 or 3 and, with the aid of other clues and auscultation, they can often be distinguished from one another. When a primary care physician is confronted with a child who has an incidental murmur that is "probably" innocent but could be organic, useful clues favoring an organic murmur are a history of congenital heart disease in a first-degree relative; a history of maternal rubella syndrome, alcohol use, or teratogenic drug use during pregnancy; a history of inappropriate sweating; a history of syncope, chest pain, or squatting; maternal diabetes mellitus; premature birth; birth at a high altitude; cyanosis; abnormal pulsations; recurrent bronchiolitis or pneumonia; chronic unexplained hoarseness; asymmetric facies with crying; and a physical appearance suggestive of a clinical syndrome.