Does Maintenance of Pulmonary Blood Flow Pulsatility at the Time of the Fontan Operation Improve Hemodynamic Outcome in Functionally Univentricular Hearts?

It is unclear whether residual anterograde pulmonary blood flow (APBF) at the time of Fontan is beneficial. Pulsatile pulmonary flow may be important in maintaining a compliant and healthy vascular circuit. We, therefore, wished to ascertain whether there was hemodynamic evidence that residual pulsatile flow at time of Fontan promotes clinical benefit. 106 consecutive children with Fontan completion (1999-2018) were included. Pulmonary artery pulsatility index (PI, (systolic pressure-diastolic pressure)/mean pressure)) was calculated from preoperative cardiac catheterization. Spectral analysis charted PI as a continuum against clinical outcome. The population was subsequently divided into three pulsatility subgroups to facilitate further comparison. Median PI prior to Fontan was 0.236 (range 0-1). 39 had APBF, in whom PI was significantly greater (median: 0.364 vs. 0.177, Mann-Whitney p < 0.0001). There were four early hospital deaths (3.77%), and PI in these patients ranged from 0.214 to 0.423. There was no correlation between PI and standard cardiac surgical outcomes or systemic oxygen saturation at discharge. Median follow-up time was 4.33 years (range 0.0273-19.6), with no late deaths. Increased pulsatility was associated with higher oxygen saturations in the long term, but there was no difference in reported exercise tolerance (Ross), ventricular function, or atrioventricular valve regurgitation at follow-up. PI in those with Fontan-associated complications or the requiring pulmonary vasodilators aligned with the overall population median. Maintenance of pulmonary flow pulsatility did not alter short-term outcomes or long-term prognosis following Fontan although it tended to increase postoperative oxygen saturations, which may be beneficial in later life.

Paediatric pulmonary hypertension and sildenafil: current practice and controversies.

In recent times, paediatric pulmonary arterial hypertension management has been transformed to focus on disease modifying strategies that improve both quality of life and survival, rather than just symptom palliation. Sildenafil, a phosphodiesterase-V inhibitor, has been at the centre of this. Despite controversial beginnings, its success in treating pulmonary arterial hypertension has led to its consideration for related pathologies such as persistent pulmonary hypertension of the newborn and bronchopulmonary dysplasia, as well as the development of a range of alternative formulations. However, this has caused its own controversy and confusion regarding the use of sildenafil in younger patients. In addition, recent data regarding long-term mortality and the repeal of US drugs approval have complicated the issue. Despite such setbacks, sildenafil continues to be a major component of the contemporary care of paediatric pulmonary hypertension in a variety of contexts, and this does not seem likely to change in the foreseeable future.

Kawasaki disease in children.

Kawasaki disease (KD) is an acute self-limiting systemic vasculitis of unknown aetiology. It is the most common cause of acquired heart disease in young children. The intense inflammatory process has a predilection for the coronary arteries, resulting in the development of aneurysmal lesions, arterial thrombotic occlusion or, potentially, sudden death. There is no specific diagnostic test; however, treatment with immunoglobulin and aspirin effectively reduces cardiac complications from 25% to 4.7% in the UK. Inflammation of the myocardium, endocardium or pericardium can occur early in the disease and endothelial dysfunction along with abnormalities of myocardial blood flow may require continuing medication, interventional catheterisation or even cardiac surgery. Several new pharmacological treatments may have important roles to play in managing KD in children and adolescents. This review discusses the history of the disease, the diagnostic challenges, epidemiology, aetiology, pathology, immunopathogenesis, treatment, genetic influences and the long-term cardiovascular sequelae.

Interventional cardiac catheterisation in congenital heart disease.

As a result of recent technological advances, more types of congenital heart disease are amenable to treatment in the cardiac catheter laboratory than ever before.1 Improved imaging techniques allow for better selection of patients, and the development of a wide range of devices specifically for use in children means that many patients can avoid surgery altogether, while those with complex congenital heart disease may require fewer or less complex surgical procedures.2 This allows for a quicker recovery and a shorter hospital stay, and gives many patients an improved quality of life in the short to medium term. However, the long term outcome for many of the newer forms of intervention is still unknown.

Acute myocardial infarction as a cause of death in palliated hypoplastic left heart syndrome.

A 20 month old child with hypoplastic left heart syndrome died suddenly from a massive myocardial infarction 15 months after a hemi-Fontan operation. This was confirmed at postmortem examination and histological examinations. The sites of surgical reconstruction were all in good condition, there were no gross anatomical coronary abnormalities, and the coronary ostia were unobstructed. On microscopy the internal coronary arteries had notable intimal and medial thickening with narrowing of the lumen, although no thrombotic occlusion was seen. To the authors' knowledge, this is the first published report of arteriosclerosis of the coronary arteries in hypoplastic left heart syndrome. It raises the question as to whether there may be a primary histological abnormality in some children with this condition or whether some mechanism of accelerated arteriosclerosis is at work.

Coronary artery changes in patients with Kawasaki disease.

Kawasaki disease (KD) is an acute, self-limiting, systemic vasculitis of unknown aetiology, which most commonly occurs in children aged 6 mo to 5 y, with a peak incidence at 9-11 mo. The inflammatory process preferentially involves the coronary arteries, potentially resulting in coronary arteritis, aneurysmal lesions, arterial thrombotic occlusion and sudden death. Kawasaki disease is the most common cause of acquired coronary vessel abnormalities in children. The cause of KD is not known, but evidence is presented for an inflammatory response and a genetic predisposition. The diagnostic tests are not yet defined, but treatment with immunoglobulin and aspirin is effective at reducing the risk of cardiac complications from 25% to 4.7% in the UK. Sequelae may occur, either acutely with myocardial, endocardial or pericardial inflammation, or many years after the original illness. There may be abnormalities of myocardial blood flow as assessed by MRI, radio-nucleide studies or echo Doppler. Such abnormalities of coronary arteries may require ongoing medication, interventional catheterization or even cardiac surgery. In the future, we hope to have more accurate diagnostic tests or prophylaxis against the disease, in addition to improved means of determining the susceptibility to or presence of long-term complications.

Coil occlusion of systemic venous collaterals in hypoplastic left heart syndrome.

OBJECTIVE: To assess the frequency of systemic venous collaterals to the atria, which may cause desaturation, after stage II reconstructive surgery for hypoplastic left heart syndrome (HLHS) and to determine whether coil occlusion prevents the need for surgical ligation. DESIGN: Prospective interventional study. SETTING: Tertiary referral centre. PATIENTS: 27 children with HLHS undergoing cardiac catheterisation between October 1996 and February 2001. INTERVENTIONS: 19 children were catheterised prestage II, 1 poststage II, and 17 prestage III. Aortic oxygen saturation (SaAo) and pulmonary artery pressure (pPA) were recorded. Angiography was performed into the left internal jugular vein to look for venous collaterals. If present, they were occluded with Cook MReye coils. Angiography was repeated to confirm occlusion, and SaAo and pPA were remeasured. RESULTS: Collaterals were found in 7 of 27 children: 1 poststage II and 6 prestage III. These were occluded with 1-3 coils without complication. Mean (SE) SaAo before occlusion was 80.2 (2.1)% in those with collaterals compared with 88.7 (1.0)% in those without (p = 0.007). There was no difference in mean pPA between the two groups. After coil occlusion mean SaAo rose to 83.8 (1.8)% (p = 0.007) and mean pPA rose from 12.5 (1.5) to 14.5 (1.8) mm Hg (p = 0.02). None required surgical ligation. CONCLUSION: Angiography should be performed at catheterisation before stage II and III surgery for HLHS to exclude systemic venous collaterals. If present, they may be safely and effectively occluded with coils to improve saturation and prevent the need for subsequent surgical ligation.