Haemoptysis is a common feature of adults with congenitally malformed hearts suffering from Eisenmenger’s syndrome. While this situation is often treated conservatively, it may not settle with such treatment. A further therapeutic option for these patients is embolisation of appropriate systemic-to-pulmonary collateral arteries. We discuss here our experience in treating two such patients with different underlying anatomical substrates.

We report the progressive development of pulmonary hypertension despite the presence of severe pulmonary stenosis in three patients with cyanotic congenital heart disease. The associated intracardiac lesions were complete transposition with a ventricular septal defect, double outlet right ventricle, and a heart with univentricular atrioventricular connection, respectively. Serial measurements on hemodynamics and histological findings documented the progression of pulmonary hypertension and pulmonary vascular diseases. The underlying etiology remains speculative. In view of the possibility of development of pulmonary hypertension despite a ‘protected’ pulmonary vasculature, corrective surgery should be contemplated as soon as technically feasible.

The life and accomplishments of Helen Taussig are reviewed at the centennial of her birth in 1898. Now, a little over 50 years since the first Blalock Taussig shunt in 1944, her legacy remains a model of brilliant scientific innovation and loving patient care.

“Faithful indeed is the spirit that remembers” (Emily Bronte, Remembrance)

Aims: Our purpose was to assess the value of magnetic resonance angiography as a non-invasive alternative to catheterization in the evaluation of patients with tetralogy of Fallot, including those with pulmonary atresia. Methods and results: We evaluated prospectively, using magnetic resonance angiography, 30 patients, aged from 1 to 18 years, 15 with tetralogy of Fallot and pulmonary stenosis, and 15 with pulmonary atresia. The studies obtained using magnetic resonance provided adequate visualization of the aorta, and provided excellent imaging of the pulmonary trunk and its right and left branches. Compared with catheterization, magnetic resonance had 100 percent sensitivity, specificity and accuracy for defining the presence or absence of the pulmonary arteries. Magnetic resonance also had 93.9 percent sensitivity, 98.2 percent specificity, and 96.7 percent accuracy for detection of stenosis or hypoplasia of the pulmonary arteries. We detected 25 major aortopulmonary collateral arteries with magnetic resonance, but only 22 with conventional angiography. There was complete agreement between the two methods in detecting patency of the arterial duct in 6 patients, and of Blalock-Taussig shunts in 12 patients. Conclusion: Magnetic resonance angiography is a useful tool in the evaluation of patients with tetralogy of Fallot. It can be considered a non-invasive alternative to cardiac catheterization in the evaluation of the pulmonary vascular anatomy.

Aims: Bosentan is efficacious in idiopathic pulmonary arterial hypertension, and the variants associated with connective tissue disease, but not currently approved for treatment of pulmonary arterial hypertension due to Eisenmenger's syndrome. We sought to evaluate its effect in adults with Eisenmenger's syndrome. Methods: We administered bosentan on the basis of compassionate use in 23 patients with Eisenmenger's syndrome, aged 37 plus or minus 14 years. Of the patients, 17 had never received specific treatment for pulmonary arterial hypertension, five were transitioned from treprostinil, and one from beraprost to bosentan. We measured functional class, saturation of oxygen, haemoglobin levels and six-minute walk distance at baseline, one, six months and at most recent follow-up. Results: Baseline functional class was IV in three, III in fifteen, and II in five patients. At follow-up, with a mean of 15 plus or minus 10 months, 13 of the 23 patients (57%) had improved by at least one functional class, from a median baseline of III to II (p equal to 0.016), mean saturation of oxygen at rest had increased from 81% to 84% (p equal to 0.001), and levels of haemoglobin had decreased from 178 plus or minus 26 grams per litre to 167 plus or minus 19 grams per litre (p equal to 0.001). Overall, the six-minute walk distance did not change from baseline of 335 metres. The distance walked by those not previously receiving specific therapy, however, improved from 318 plus or minus 129 to 345 plus or minus 123 metres (p equal to 0.03). Conclusion: Treatment of adults with Eisenmenger's syndrome using bosentan significantly improved functional class, saturation of oxygen at rest, and decreased levels of haemoglobin. Treatment with bosentan was associated with improvement in six-minute walk distance in those not previously receiving specific therapy. In patients already in receipt of specific therapy, transition to bosentan resulted in no clinical deterioration.

Aims: The purpose of our study was to ascertain the outcome of the construction of a Blalock–Taussig shunt in patients aged 12 years and over. Patients and methods: We identified 21 patients in whom a Blalock–Taussig shunt had been constructed subsequent to the age of 12 years. Of the patients, 9 were female, and their median age was 18.5 years, with a range from 12 to 46 years. All had usual atrial arrangement, and the atrioventricular connections were concordant in 11, with univentricular atrioventricular connection in 10. Pulmonary atresia was present in 8 (38 per cent), and pulmonary stenosis, either valvar or subvalvar, in 13. An interposition graft had been placed between the subclavian and pulmonary arteries in 16 patients, and an end-to-side anastomosis between the arteries in 5. Results: One patient had died in hospital, while 4 patients had died during the period of follow-up after initial construction of the shunt. Long-term follow-up was available in 86 per cent of patients. In the 3 patients lost to follow-up, the shunt had been known to be functioning at periods of 4, 8, and 10 years, respectively. Actuarial freedom from death after a period of 17 years was 76 per cent. In 8 patients, a period of 10 years had elapsed with the shunt patent, and a further 10 had a patent shunt after 5 years follow-up. Symptomatic improvement was reported in 16 (76 per cent) patients, although adverse cardiac events had occurred during follow-up in 17, including congestive heart failure in 3, atrial fibrillation in 3, and endocarditis in 2. In 2 patients, it had been possible to proceed to biventricular repair, one with tetralogy of Fallot, and the other having a Rastelli procedure. Further in 3 patients, it had been possible to construct the Fontan circulation, or one of its variants. One patient has undergone cardiac transplantation, while 2 are awaiting transplantation. Conclusions: Symptomatic improvement can be achieved by construction of a Blalock–Taussig shunt in older subjects, and the risks of surgery are low. Later repair may be feasible in some patients, but adverse cardiac events may follow the increased volume load on the systemic ventricle.