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Kawasaki disease (KD) is an inflammatory disorder of young children, associated with vasculitis of the coronary arteries with subsequent aneurysm formation in up to one-third of untreated patients. Those who develop aneurysms are at life-long risk of coronary thrombosis or the development of stenotic lesions, which may lead to myocardial ischaemia, infarction or death. The incidence of KD is increasing worldwide, and in more economically developed countries, KD is now the most common cause of acquired heart disease in children. However, many clinicians in the UK are unaware of the disorder and its long-term cardiac complications, potentially leading to late diagnosis, delayed treatment and poorer outcomes. Increasing numbers of patients who suffered KD in childhood are transitioning to the care of adult services where there is significantly less awareness and experience of the condition than in paediatric services. The aim of this document is to provide guidance on the long-term management of patients who have vascular complications of KD and guidance on the emergency management of acute coronary complications. Guidance on the management of acute KD is published elsewhere. [ABSTRACT FROM AUTHOR]

Objective: Kawasaki disease (KD) is an increasingly common vasculitis with risk of coronary artery aneurysms (CAAs). The last UK survey was in 1990, whereas current epidemiology, treatment patterns and complication rates are unknown. The aim of this study was to address this knowledge gap.Methods: A British Paediatric Surveillance Unit survey in the UK and Ireland from 1 January 2013 to 28 February 2015 ascertained demographics, ethnicity, seasonal incidence, treatment and complication rates.Results: 553 cases were notified: 389 had complete KD, 46 had atypical KD and 116 had incomplete KD; 2 were diagnosed at postmortem with an incidence of 4.55/100 000 children under 5 years, with a male to female ratio of 1.5:1 and a median age of 2.7 years (2.5 months-15 years). Presentation was highest in January and in rural areas. Most were white (64%), and Chinese and Japanese Asians were over-represented as were black African or African mixed-race children. 94% received intravenous immunoglobulin (IVIG). The overall CAA rate was 19%, and all-cardiac complications affected 28%. Those with CAA received IVIG later than in those without (median 10 days vs 7 days). Those under 1 year had fewer symptoms, but the highest CAA rate (39%). Overall 8 of 512 cases (1.6%) had giant CAA, and 4 of 86 cases (5%) under 1 year of age developed giant CAA. Mortality from KD was 0.36%.Conclusions: The UK and Ireland incidence of KD has increased and is more frequently seen in winter and rural areas. Delayed IVIG treatment is associated with CAA, suggesting earlier and adjunctive primary treatment might reduce complications to prevent CAA, particularly in the very young. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Respiratory syncytial virus (RSV) is a common infection, causing bronchiolitis in over 70% of infants each year and almost all children by the age of 2. It is the leading cause of acute lower respiratory tract infections and admissions to hospital worldwide. Previously healthy infants may have a prolonged cough or wheezing following RSV infection but up to 20% of those with congenital cardiac disease will be hospitalized and have significant morbidity and mortality. For this reason, it is generally recommended that many such infants should receive prophylaxis against RSV infection with palivizumab. This paper reviews the current state of knowledge about RSV affecting children with congenital heart disease from the perspective of those living in the UK and the current protection offered to such children. We also discuss the plans for the future of protection against RSV. [ABSTRACT FROM AUTHOR]

Echocardiography is the mainstay in screening for pulmonary hypertension (PH). International guidelines suggest echocardiographic parameters for suspecting PH, but these may not apply to many adults with congenital heart disease (ACHD). PH is relatively common in ACHD patients and can significantly affect their exercise capacity, quality of life, and prognosis. Identification of patients who have developed PH and who may benefit from further investigations (including cardiac catheterization) and treatment is thus extremely important. A systematic review and survey of experts from the United Kingdom and Ireland were performed to assess current knowledge and practice on echocardiographic screening for PH in ACHD. This paper presents the findings of the review and expert statements on the optimal approaches when using echocardiography to assess ACHD patients for PH, with particular focus on major subgroups: patients with right ventricular outflow tract obstruction, patients with systemic right ventricles, patients with unrepaired univentricular circulation, and patients with tetralogy of Fallot with pulmonary atresia. [ABSTRACT FROM AUTHOR]

Background: Coronary artery aneurysms resulting from unrecognised or untreated Kawasaki Disease (KD) have thrombotic and stenotic potential leading to myocardial infarction. We aimed to characterise the prevalence and outcomes of patients presenting to a UK-based adult tertiary cardiology service with angiographic evidence of aneurysm or ectasia., Methods: Retrospective review was undertaken of reports and original coronary angiograms in all patients under 50years old undergoing coronary angiography between 2011 and 2015. Aneurysm/ectasia were defined by calibre discrepancy >1.5× and the number of segments involved. Aneurysms were categorised as being probable, or unlikely to represent unrecognised KD., Results: Aneurysm or ectasia was observed in 49 of 1578 patients (3.7%). Eleven patients had angiographic findings consistent with probable antecedent KD (0.8%), a further 18 patients had evidence of coronary artery aneurysm (1.4%) and ectasia was observed in 20 patients (1.5%). The commonest mode of presentation was ST elevation myocardial infarction, observed in 71.4% of patients. Review of angiogram written reports demonstrated a spurious application of the terms aneurysm and ectasia, with a sensitivity of 10.3% & 55.0%, and a positive predictive value of reporting of 50.0% & 52.4%, respectively., Conclusions: As the first UK study to characterise the angiographic prevalence of Kawasaki Disease, both aneurysm and ectasia were observed with a relatively low frequency. However, their presence is associated with high-risk acute ischaemic presentations. Furthermore, we demonstrated a poor level of reporting of coronary abnormalities and advocate an increased awareness of Kawasaki disease and coronary aneurysm/ectasia amongst adult cardiologists., (Copyright © 2017 Elsevier Ireland Ltd. All rights reserved.)

Background: Pulmonary hypertension is a condition of complex aetiology that culminates in right heart failure and early death. Soluble guanylate cyclase (sGC) stimulators are a promising class of agents that have recently gained approval for use., Objectives: To evaluate the efficacy of sGC stimulators in pulmonary hypertension., Search Methods: We searched CENTRAL (Cochrane Central Register of Controlled Trials), MEDLINE, EMBASE and the reference lists of articles. Searches are current as of 12 February 2016., Selection Criteria: We selected randomised controlled trials (RCTs) involving participants with pulmonary hypertension of all ages, severities and durations of treatment., Data Collection and Analysis: AW, MS and RW independently selected studies, assessed evidence quality and extracted data. This process was overseen by RT and SG. All included studies were sponsored by the drug manufacturer., Main Results: Five trials involving 962 participants are included in this review. All trials were of relatively short duration (< 16 weeks). Due to the heterogenous aetiology of pulmonary hypertension in participants, results are best considered according to each pulmonary hypertension subtype.Pooled analysis shows a mean difference (MD) increase in six-minute walking distance (6MWD) of 30.13 metres (95% CI 5.29 to 54.96; participants = 659; studies = 3). On subgroup analysis, for pulmonary arterial hypertension (PAH) there was no effect noted (6MWD; MD 11.91 metres, 95% CI -44.92 to 68.75; participants = 398; studies = 2), and in chronic thromboembolic pulmonary hypertension (CTEPH) sGC stimulators improved 6MWD by an MD of 45 metres (95% CI 23.87 to 66.13; participants = 261; studies = 1). Data for left heart disease-associated PH was not available for pooling. Importantly, when participants receiving phosphodiesterase inhibitors were excluded, sGC stimulators increased 6MWD by a MD of 36 metres in PAH. The second primary outcome, mortality, showed no change on pooled analysis against placebo (Peto odds ratio (OR) 0.57, 95% CI 0.18 to 1.80).Pooled secondary outcomes include an increase in World Health Organization (WHO) functional class (OR 1.53, 95% CI 0.87 to 2.72; participants = 858; studies = 4), no effect on clinical worsening (OR 0.45, 95% CI 0.17 to 1.14; participants = 842; studies = 3), and a reduction in mean pulmonary artery pressure (MD -2.77 mmHg, 95% CI -4.96 to -0.58; participants = 744; studies = 5). There was no significant difference in serious adverse events on pooled analysis (OR 1.12, 95% CI 0.66 to 1.90; participants = 818; studies = 5) or when analysed at PAH (MD -3.50, 95% CI -5.54 to -1.46; participants = 344; studies = 1), left heart disease associated subgroups (OR 1.56, 95% CI 0.78 to 3.13; participants = 159; studies = 2) or CTEPH subgroups (OR 1.29, 95% CI 0.65 to 2.56; participants = 261; studies = 1).It is important to consider the results for PAH in the context of a person who is not also receiving a phosphodiesterase-V inhibitor, a contra-indication to sGC stimulator use. It should also be noted that CTEPH results are applicable to inoperable or recurrent CTEPH only.Evidence was rated according to the GRADE scoring system. One outcome was considered high quality, two were moderate, and eight were of low or very low quality, meaning that for many of the outcomes the true effect could differ substantially from our estimate. There were only minor concerns regarding the risk of bias in these trials, all being RCTs largely following the original protocol. Most trials employed an intention-to-treat analysis., Authors' Conclusions: sGC stimulators improve pulmonary artery pressures in people with PAH (who are treatment naive or receiving a prostanoid or endothelin antagonist) or those with recurrent or inoperable CTEPH. In these settings this can be achieved without notable complication. However, sGC stimulators should not be taken by people also receiving phosphodiestase-V inhibitors or nitrates due to the risks of hypotension, and there is currently no evidence supporting their use in pulmonary hypertension associated with left heart disease. There is no evidence supporting their use in children. These conclusions are based on data with limitations, including unavailable data from two of the trials.

An anomalous left coronary artery from the pulmonary artery (ALCAPA) is rarely associated with persistent ductus arteriosus (PDA). A large PDA can maintain perfusion in the left coronary artery, delaying presentation. Assessing the origin of the coronary arteries before PDA ligation is difficult, often being performed in very small or even preterm babies. We present the case of a 5-month-old infant with echocardiographic features of mitral regurgitation and subendocardial ischemia who experienced ischemia and cardiac arrest after PDA ligation. Transesophageal echocardiography demonstrated ALCAPA, and left coronary translocation was performed. The infant was discharged after 10 days., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Children with pulmonary hypertension routinely undergo pulmonary vascular resistance studies to assess the disease severity and vasodilator responsiveness. It is vital that results are accurate and reliable and are not influenced by the choice of anaesthetic agent. However, there are anecdotal data to suggest that propofol and inhalational agents have different effects on pulmonary vascular resistance., Methods: A total of 10 children with pulmonary hypertension were selected sequentially to be included in the study. To avoid confounding because of baseline anatomic or demographic details, a crossover protocol was implemented, using propofol or isoflurane, with time for washout in between each agent and blinding of the interventionalist., Results: Pulmonary and systemic vascular resistance were not significantly different when using propofol or isoflurane. However, the calculated resistance fraction - ratio of pulmonary resistance to systemic resistance - was significantly lower when using propofol than when using isoflurane., Conclusions: Although no difference in pulmonary vascular resistance was demonstrated, this pilot study suggests that the choice of anaesthetic agent may affect the calculation of relative pulmonary and systemic vascular resistance, and provides some preliminary evidence to favour propofol over isoflurane. These findings require replication in a larger study, and thus they should be considered in future calculations to make informed decisions about the management of children with pulmonary hypertension.

Pulmonary hypertension (PHT) is a well recognised feature of untreated congenital heart disease. This article will review the causes, known mechanisms, appropriate investigations and current therapies for PHT. The reader will understand the difference between PHT due to high pulmonary blood flow and PHT that is due to high pulmonary vascular resistance. The former is best treated by surgical or catheter intervention, whereas for the latter (Eisenmenger syndrome) only palliation is possible with medication or transplantation. Echocardiography and electrocardiography (ECG) should be performed in any child where there is a possibility of pulmonary hypertension, especially with long standing chronic lung disease and minor left to right shunt. Often these children may have dual pathology and their investigation and management may be a complex interaction between cardiac and respiratory therapists. New treatments and new techniques of assessment are now available and this may lead to improved recognition of PHT and prevention of long term disability as a result.

Objective: To document the echocardiographic features of tetralogy of Fallot during fetal and postnatal life. Correlation of echocardiographic findings with the requirement for early intervention prior to definitive repair., Design: Retrospective observational study., Setting: A tertiary fetal cardiology unit., Patients: Fetuses with a diagnosis of tetralogy of Fallot identified from a prospective database between 1 January 1999 and 31 October 2002. MAIN MEASURES OF OUTCOME: Growth of aorta and pulmonary trunk during fetal and postnatal life. Doppler assessment of the great arteries both prenatally and postnatally. Clinical outcome to definitive repair., Results: We identified 25 fetuses with tetralogy of Fallot, 23 having a pulmonary valvar diameter below the normal range at some point during gestation. The ratio of the diameter of the aortic to the pulmonary valve was abnormal in all cases. The pulmonary arterial Doppler velocity was within the normal range in six fetuses at presentation,and elevated in the remainder. In two fetuses, the right ventricular outflow tract was patent during fetal life, but had become atretic at birth. Both of these fetuses had reversal of flow in the arterial duct at presentation during fetal life. In 2 fetuses in whom we showed poor growth of the pulmonary trunk in late gestation, it was necessary to intervene early. The Doppler velocity across the pulmonary valve during fetal life did not differentiate between babies who required early intervention and those who were repaired electively. There was a marked increase in pulmonary arterial Doppler velocity following birth, which became more elevated with age. Of 18 liveborn infants, 17 have survived, with 2 having balloon dilation of the right ventricular outflow tract, and 3 insertion of a Blalock-Taussig shunt prior to definitive repair., Conclusions: In tetralogy of Fallot, features of pulmonary valvar hypoplasia and obstruction are evident during fetal life. Progression of obstruction in the right ventricular outflow tract was observed during fetal life as well as postnatally. Reversal of flow in the arterial duct, and failure of growth of the pulmonary trunk, predicted the need for early surgery to maintain pulmonary blood flow. Parents should be counselled about the possibility of emergency intervention being required after birth. Affected fetuses should be delivered at units with experience of managing the cyanosed newborn.