We describe the importance of angiographic identification of collateral venous channels by balloon occlusion venography after bidirectional cavopulmonary connections. Use of the balloon occlusion technique is essential for identification of these vessels, as they can easily be missed by standard venous angiography, with possible clinical consequences for postoperative management.

Two neonates with severe pulmonary stenosis deteriorated immediately after successful balloon valvoplasty as a result of increased infundibular obstruction. They were treated with β-adrenoceptor blockers and intravascular expansion with limited success. Phentolamine was then given, resulting in dramatic improvement. The children could be weaned from the ventilator within hours of starting this new therapeutic modality.

A patient with unrepaired complex pulmonary atresia had a normal life, achieving two successful pregnancies, until the age of 44 years. Confluent central pulmonary arteries were supplied by a fistu-lous communication from the left coronary artery, and from other collateral arteries arising from the underside of the aortic arch. Unusual aneurysms were present. Death at the age of 46 resulted from dissection and rupture of an aneurysmal dilation of the pulmonary trunk.

Doppler echocardiography allows accurate serial assessment of pulmonary valvar stenosis by measuring the velocity of the jet stream through the pulmonary valve. Between 1979 and 1997, we saw 174 patients with isolated pulmonary valvar stenosis. At admission their ages ranged from 9 days to 22.5 years. We measured the velocity over the pulmonary valve, and the thickness of the anterior wall of the right ventricle, and made a study of their electrocardiograms. We found that rapid increases and decreases occurred in almost every age-group. For patients with a trivial, mild or moderate level of stenosis, severe stenosis developed in 3, 10 and 9%, respectively. In most of the patients, 122 (90%), in whom there was more than one examination, a change in pressure gradient between −12mmHg/year and +3mmHg/year was found. Only 7 patients had an increase of more than 10mmhg per year. In contrast with our patients having aortic stenosis, these with stenosis of the pulmonary valve showed no rapid increase in early childhood. Indeed, in 58% the severity of the stenosis decreased. No correlation was found when comparing the echocardiographic measurements of the thickness of the anterior wall of the right ventricle with the voltages on the electrocardiogram. A significant relation was found however, between an increasing pressure gradient and thickened valvar leaflets (p = 0.017).

A clinical study on the outcomes of Glenn anastomoses performed since 1987 in eight consecutive patients aged ≥16 years, and in two performed earlier, showed poor results. One badly selected patient died early as a consequence of high venous pressure, while a further seven had early complications. Seven of eight hospital survivors were followed for 1–10 (median 4.2) years with two deaths (1 and 4 years later). Of the remaining five patients, two improved temporarily, but increased arterial oxygen saturation was not maintained after 6 months. The two patients who had undergone a Glenn anastomosis 10 and 34 years earlier were shown to have pulmonary arteriovenous fistulas. The Glenn anastomosis in these older patients is associated with high rates of complication and appears not to give adequate palliation, particularly when it is the only source of pulmonary blood supply. In the adult, the Glenn anastomosis can be used as a staging procedure for Fontan-type surgery, but must be combined with another source of pulmonary arterial supply. Any adult having a Glenn anastomosis, particularly without another source of pulmonary arterial supply, should be warned of the possibility of worsening of cyanosis and symptoms. The second stage of the procedure may need to be performed soon after the first should the hypoxia prove intolerable.

Recent studies of intravascular ultrasound of the pulmonary arteries suggest that the technique can detect intimal and medial thickening in patients with pulmonary hypertension, potentially providing a method of assessing severity of pulmonary vascular disease in life. A major drawback of the technique is that only the elastic pulmonary arteries are accessible to current ultrasound catheters. The aim of this study was to determine whether morphological changes in vessels accessible to intravascular imaging reflect severity of pulmonary vascular disease and are of a sufficient degree to be detectable by current ultrasound catheters. Morphometric studies of the elastic pulmonary arteries were performed in specimens of lungs from 24 patients who had died with pulmonary hypertension (aged 3 weeks–9 years) and compared with measurements from infants who had died from sudden infant death syndrome. Morphological changes evident in the elastic pulmonary arteries in pulmonary hypertension included luminal dilation and medial thickening but these changes were too variable to be predictive of the severity of peripheral pulmonary vascular disease. Intimal thickening and atherosclerosis were present only in those with advanced pulmonary hypertensive disease. The changes of medial thickening and luminal dilation, nonetheless, are too variable to be predictive of the severity of peripheral pulmonary vascular disease. Thus, imaging by intravascular ultrasound may help confirm advanced pulmonary vascular disease, but is unlikely to differentiate less severe pulmonary vascular disease when the patient may still potentially be operable.

Controversy remains about the growth of the pulmonary arteries following a bidirectional superior cavopulmonary anastomosis in children with complex cyanotic congenital cardiac malformations. This is partially due to the morphological heterogeneity of the patients, and partially due to methodological differences in series published so far. It is further complicated by the variable use, in different centres, of additional sources of pulmonary blood flow. We believe that the fate of these arteries preoperatively is significantly influenced by the amount of pulmonary blood flow and the initial size of the arteries. Separate assessment of the pulmonary arterial development postoperatively is recommended for those who, initially, had relatively small as opposed to larger than normal pulmonary arteries. Measurement of the diameters of both pulmonary arteries just prior to their first point of branching, together with the use of Z-score evaluation rather than the Nakata index, is discussed. It remains to be established whether, over time, the bidirectional cavopulmonary anastomosis is effective in developing adequately the pulmonary arteries in preparation for an ultimate total cavopulmonary connection, or even as isolated long-term palliation. A prospective, multi-institutional study involving sequential non-invasive assessment of pulmonary arterial development (using, for example, magnetic resonance imaging) is required to solve the outstanding problems.

In this report, we describe our experience with transcatheter occlusion of congenital coronary arterial fistulas in adults. From November 1992 to November 1996, 5 symptomatic patients, aged from 47 to 70 years, underwent transcatheter occlusion of fistulas using a retrograde arterial approach. All had chest pain or dyspnea on exertion. Detachable balloons were used in 4 patients, and Gianturco coils in 1. Detachable balloons were implanted through a Debrun system, while the coils were implanted through a 5 French right coronary Judkins catheter. Both were passed through an 8 French guiding catheter (Amplatz II). Each patient had a single fistula. The fistulas originated from the right coronary artery in 3 patients, and from the circumflex artery in 2. They drained into the pulmonary trunk in 3 patients, into the right atrium in 1, andinto a bronchial artery in the other. All fistulas were occluded completely in the catheterization laboratory, and the procedures were uncomplicated. At follow up, 3 patients underwent coronary angiography, and there was no evidence of recanalization. Transcatheter embolization in adults of single congenital coronary fistulas with detachable balloons and coils is safe and effective and can be regarded as an acceptable alternative to surgery.