The origins of the Ross operation

These historic pictures were taken at the time of the operation in 1961 by Donald Ross who gave them to me. Photography was routine for cardiac surgery in Guys Hospital at this time as Sir Russell Brock was insistent that appearances were accurately recorded as well as described in operation reports.peer-reviewe

The chest x-ray in congenital heart disease 5

This paper is the fourth of the series focusing on the chest x-ray (CXR). The patient in question was a woman aged 56 years who was sent to a cardiologist with left chest pain that was of non-cardiac origin.peer-reviewe

Understanding Empowerment Through Virtue Theory

Applying virtue theory to empowerment, domination, and oppression

702–6 Eisenmenger's Syndrome and Progressive Pulmonary Hypertension After Defect Closure: Prognosis and Cause of Death

The possibility of transplantation for patients with pulmonary hypertension makes it important to predict prognosis and causes of death. This studies the state (Ability Index [Al]), complications, age at and cause of death of 143 patients with Eisenmenger Syndrome (E Group) due to VSD (28%), one ventricle (9%), A-V canal (8%) and truncus (8%), 50 of whom died during observation and 19 patients whose defects (VSD, duct, truncus) were repaired in childhood and who died from progressive pulmonary hypertension (PPH Group). In E Group, 8 deaths occurred before age 16 years (child in UK); only 3 “natural” deaths, the other 5 early in relation to cardiac surgery. 42 of E Group died age 19–57 years, the majority in the 3rd decade from right heart failure (24%), sudden (26%), cerebral (14%), haemoptysis (14%), post car-diothoracic intervention (14%), and extracardiac surgery (7%). 22 (24%) of 93 alive in E Group survived to age 40–58 years with Al 2 (well, 13) and 3/4 (9). The PPH Group died age 2–35 years, 75% before 20 years. Prognosis in the PPH Group is worse than Eisenmengers where death in childhood is uncommon. In both groups frank heart failure and exertional presyncope are bad prognostic features but commoner in the PPH Group. In Eisenmengers, any surgery is dangerous. Large haemoptysis heralded disaster in 50% with symptoms when due to right pulmonary artery thrombosis. With the better prognosis of Eisenmenger cf. PPH, it is important to recognize those whose pulmonary vascular resistance may rise after surgery and will thus have an added risk for lung transplantation

The chest x-ray in congenital heart disease 7

This paper is the seventh of the series focusing on the chest x-ray (CXR). This CXR shows a normal vascular pedicle with a left aortic knuckle and normal pulmonary vascularity. There is some prominence of the apex to the left with a rounded contour suggestive of left ventricular enlargement. There is no evidence of either atria being abnormally placed or distorted. An interesting point is that the patient has an aortic valve replacement and the disk valve prosthesis lies in an unusual and high horizontal position. This suggests that aortic valve is in unusual position such as in transposition of great arteries following an arterial switch procedure. This is the case in this patient wherein aortic valve replacement was carried out for aortic regurgitation.peer-reviewe

Total anomalous pulmonary venous drainage and coarctation of the aorta

The chest x-ray (CXR) is a useful investigation in congenital heart disease, and has been probably ignored in recent years as echocardiography and other exotic and expensive imaging techniques can show anatomy and physiology. The CXR is a simple, quick and cheap test that yields useful diagnostic information, heart size, lung pathology as well as providing a readily available documentation of these facts for serial comparison. Such information is useful and even vital for the paediatric and the grown up congenital heart population. The echocardiogram gives little or no information on lung pathology or secondary pulmonary effects. The CXR readily demonstrates the classic heart shapes of congenital heart disease and supplies an easily seen record of heart size with low radiation levels. For young clinicians to find teaching about this cheap investigation is hard. This article is the first of a series of articles that highlight the use and the value of the CXR in congenital heart disease.peer-reviewe

Recurrent Pneumonia and a Normal Heart: Late Complication after Repair of Hemianomalous Pulmonary Venous Drainage—A Cautionary Tale

Hemianomalous pulmonary venous drainage with intact atrial septum is a rare congenital anomaly and reports of its surgical repair and the long-term complications related to the correction are only infrequently encountered in the literature. We report the case of a patient with hemianomalous pulmonary venous drainage and intact atrial septum who underwent surgical repair using a pericardial baffle and creation of an “atrial septal defect” aged 15 years. Dyspnoea and recurrent chest infections started 7 months after surgery when he was seen by a respiratory physician without cardiac followup. He presented again aged 28 years with a recurrent pneumonia investigated over 6 weeks and heart pronounced normal from examination and echocardiography. Correct diagnosis was made in Grown Up Congenital Heart (GUCH) clinic stimulating review of data and catheterisation with pulmonary artery angiography which confirmed it. We feel that this case highlights the importance of specialist care and followup for GUCH patients

A service for grown up patients with congenital heart disease (GU CH) in Malta

Untreated congenital heart disease takes a heavy toll for those born with such anomalies, with approximately 65% dying in infancy and 15-20% reaching adolescence and adulthood. J Malta has had the same incidence of congenital heart disease in live births as other European countries, i.e. 0.8/ 1000 live births 2 It has been fortunate to be able to give children optimal therapy by the ability to send infants and children to London for treatment including open heart surgery over the last 40 years. The majority have returned improved or cured, with diminishing mortality rates (figure 1) and increasing survival rates in the region of 95%.3 Many have passed adolescence becoming parents and even grandparents, and are or have been in full employment, contributing to the community.peer-reviewe

Presentation and attrition in complex pulmonary atresia

AbstractObjectives. This study summarized patterns of presentation and attrition in complex pulmonary atresia.Methods. We performed a retrospective review of age at presentation, referral source, pulmonary artery and collateral anatomy and surgical history of 218 patients from two institutions dealing with congenital heart disease throughout life.Results. Approximately 65% of pulmonary atresia appears in infancy, with 50% of patients severely symptomatic from cyanosis and 25% from heart failure. Compared with those presenting undiagnosed, patients referred secondarily for specialist management trend tobe older whe first seen, and cre must be taken when generalizing about the natural history of the condition from their survival experience. Overall actuarial survival, including the effects of operation, suggests that 60% (95% condifence limits [CL] 43 to 73) of patients presenting in infancy survice to their first birthday, 65% (95% CL to 51 to 74) those alive at 1 year old survive to the age of 10, and 16 (95% CL 5 to 31) of those alive at 10 years old survive to age 35.Conclusions. Novel surgical approaches habve generally been applied beyond infancy in patients selected by their survival through the period of greatest attrition for this disease. Unless successful application in symptomatic infants is demonstrated, we cannot assume that these serial and complicated operations will have a major impact on the outlook of most patients with complex pulmonary atresia

Effects of Chronic Hypoxemia on Chemosensitivity in Patients With Univentricular Heart

AbstractObjectives. We 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.Background. Patients with univentricular heart demonstrate excessive ventilation during exercise. Chronic hypoxemia may alter chemoreceptor function, affecting ventilation.Methods. Cardiopulmonary 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% CO2in 93% O2, respectively.Results. Peak O2consumption 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 CO2output, 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 O2saturation, 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 CO2tension and thus may partly explain the increased exercise ventilatory response in this group.Conclusions. Hypoxic chemosensitivity is blunted in patients with univentricular heart and cyanosis and does not determine the exercise ventilatory response. CO2elimination appears more important. The blunting of hypoxic chemosensitivity is reversible once chronic hypoxemia is relieved, as evident in the Fontan group