Treatment of pediatric pulmonary hypertension

Pulmonary hypertension was once thought to be a rare condition and only managed in specialized centers. Now however, with the advent of echocardiography, it is found in many clinical scenarios, in the neonate with chronic lung disease, in the acute setting in the intensive care unit, in connective tissue disease and in cardiology pre- and postoperatively. We have a better understanding of the pathological process and have a range of medication which is starting to be able to palliate this previously fatal condition. This review describes the areas that are known in this condition and those that are less familiar. The basic physiology behind pulmonary hypertension and pulmonary vascular disease is explained. The histopathologic process and the various diagnostic tools are described and are followed by the current and future therapy at our disposal

Kawasaki disease and coronary artery aneurysms:From childhood to adulthood

Kawasaki disease is an acute, systemic vasculitis of childhood and confers a 25% risk of developing coronary artery aneurysms. Its etiology is unknown, but the incidence is increasing rapidly with linked gene polymorphisms having been identified. A constellation of symptoms, epidemics and seasonality all implicate an unidentified infective or environmental cause. Intravenous immunoglobulin therapy, aspirin and steroids all form the mainstay of acute treatment and reduces the incidence of coronary artery aneurysms if given before 7 days. However, in some, these lesions persist and require ongoing management during follow-up during childhood and into adult life. Evidence for further investigations in order to minimize complications is presented in order to minimize the myofibroblast proliferation and stenosis in the long term.</jats:p

Pulmonary hypertension in congenital heart disease

Pulmonary hypertension is defined as a mean pulmonary arterial pressure ≥25 mmHg. We focus on its relevance in congenital heart disease, reviewing pathophysiology, diagnosis and management. Pulmonary hypertension is a relatively common complication of congenital heart disease, with adult prevalence between 5 and 10%. A multifactorial cause is recognized, relating to the size and nature of cardiac defect as well as environmental and genetic factors. More complex disease is increasingly recognized rather than pure Eisenmenger complex. Remodeling of the pulmonary vascular bed causes increased pulmonary vascular resistance diagnosed by a collection of investigations including echocardiography, exercise testing, cardiac catheterization, MRI and CT scanning. Management employs disease-modifying medications which are now used with increasing benefit. </jats:p

Management of Adults With Congenital Heart Disease and Pulmonary Arterial Hypertension in the UK:Survey of Current Practice, Unmet Needs and Expert Commentary

BACKGROUND: Pulmonary arterial hypertension (PAH) is a well-recognised complication of adult congenital heart disease (CHD). However, management is not currently standardised between centres and specific guidelines are lacking. In order to identify and understand the unmet needs related to PAH associated with CHD (PAH-CHD), a survey of physicians was performed. METHODS: An electronic survey was sent to two physician groups: (1) cardiologists registered in a UK cardiology directory; (2) specialist pulmonary hypertension (PH) physicians known to manage patients with adult PAH-CHD. The questions related to referral pathways, screening, therapy and palliative care. RESULTS: 821 surveys were distributed and 106 were returned. Respondents included a broad mix of specialist physicians with many patients along with general cardiologists managing only a small number of PAH-CHD patients. Although 97% of respondents have access to a specialist PH centre, patients are still being managed in non-specialist settings. Shared care arrangements are widespread but only 41% have formal shared care protocols. Palliative care services are limited and general cardiologists rarely perform 6-minute walk tests (6MWT) or quality of life assessments. People with PAH-CHD are often undertreated, with 39% of respondents reporting that fewer than 25% of these patients were receiving PAH-specific therapies. CONCLUSIONS: The survey revealed gaps and inconsistencies in the management of patients with PAH-CHD therefore patient-specific guidance is needed for many of these aspects

Giant coronary artery aneurysms in a 12-week-old infant with incomplete Kawasaki disease

Kawasaki disease (KD) is an acute inflammatory vasculitis that occurs worldwide and disproportionately affects male children, most commonly between the ages of 6 months and 5 years. KD can present with only a few features and thus be difficult to diagnose, particularly in the youngest and oldest patients. We describe a 12-week-old Caucasian female infant who presented with rash and fever but no other features of KD, who developed giant coronary artery aneurysms. Considering how common is the presentation of a febrile infant with a rash, this case highlights the importance of considering KD early in the differential diagnosis for any infant with unexplained fever. Furthermore, it emphasises how echocardiography can help in the investigation of a febrile child with no clear source of infection.This case report is freely available online via Open Access. Click on the Additional Link above to access it via the publisher's site

Outcomes following aortic valve procedures in 201 complex congenital heart disease cases:results from the UK National Audit

OBJECTIVES: Some patients with complex congenital heart disease (cCHD) also require aortic valve (AoV) procedures. These cases are considered high risk but their outcome has not been well characterized. We aim to describe these scenarios in the current practice, and provide outcome data for counselling and decision-making. METHODS: This was a retrospective study using the UK National Congenital Heart Disease Audit data on cCHD patients undergoing aortic valve replacement, balloon dilation (balloon aortic valvuloplasty) or surgical repair (surgical aortic valve repair) between 2000 and 2012. Coarsened exact matching was used to pair cCHD with patients undergoing AoV procedures for isolated valve disease. RESULTS: A total of 201 patients with a varied spectrum of cCHD undergoing 242 procedures were included, median age 9.4 years (1 day–65 years). Procedure types were: balloon aortic valvuloplasty (n = 31, 13%), surgical aortic valve repair (n = 57, 24%) and aortic valve replacement (n = 154, 63%). Mortality at 30 days was higher in neonates (21.8% vs 5.3%, P = 0.02). Survival at 10 years was 83.1%, freedom from aortic valve replacement 83.8% and freedom from balloon aortic valvuloplasty/surgical aortic valve repair 86.3%. Neonatal age (P < 0.001), single ventricle (P = 0.08), concomitant Fontan/Glenn (P = 0.002) or aortic arch procedures (0.02) were associated with higher mortality. cCHD patients had lower survival at 30 days (93% vs 100%, P = 0.003) and at 10 years (86.4% vs 96.1%, P = 0.005) compared to matched isolated AoV disease patients. CONCLUSIONS: AoV procedures in cCHD can be performed with good results outside infancy, but with higher mortality than in isolated AoV disease. Neonates and patients with single ventricle defects, especially those undergoing concomitant Fontan/Glenn, have worse outcomes