The Paediatric Cardiology Hall of Fame ­ Donald Nixon Ross.

Donald Nixon Ross, FRCS (4 October 1922 to 7 July 2014) was a South African-born British cardiothoracic surgeon, who developed the pulmonary autograft, known as the Ross procedure, for the treatment of aortic valve disease, and also performed the first heart transplant in the United Kingdom in 1968. This paper, written by Jane Somerville, Professor of Cardiology [Retired], Imperial College London, London, United Kingdom, provides the personal recollections about Donald Ross from Jane Somerville, and thus provides a unique snapshot of cardiac surgical history.

The World Congress of Paediatric Cardiology and Cardiac Surgery: a short factual history by Jane Somerville.

The World Congress of Paediatric Cardiology and Cardiac Surgery has survived with minimal assets and simple organisation. Each congress is special, taking on the humour, flavour, and culture of the organising country. It is hard work for a few organisers and money is hard to raise. The steering committee works closely, fairly, and successfully, and even though accused of being secretive and effete that does not matter. It is efficient and produces successful, happy world congresses, where all involved with the speciality are welcome. With so many "grown-ups" with congenital heart disease, it is no longer just a paediatric problem - maybe the name of this congress must change again. Regardless, the flag must fly on.

Parallel randomized controlled feasibility trials of the "Active Brains" digital intervention to protect cognitive health in adults aged 60-85.

Introduction: Multidomain interventions to address modifiable risk factors for dementia are promising, but require more cost-effective, scalable delivery. This study investigated the feasibility of the "Active Brains" digital behavior change intervention and its trial procedures. Materials and methods: Active Brains aims to reduce cognitive decline by promoting physical activity, healthy eating, and online cognitive training. We conducted 12-month parallel-design randomized controlled feasibility trials of "Active Brains" amongst "lower cognitive scoring" (n = 180) and "higher cognitive scoring" (n = 180) adults aged 60-85. Results: We collected 67.2 and 76.1% of our 12-month primary outcome (Baddeley verbal reasoning task) data for the "lower cognitive score" and "higher cognitive score" groups, respectively. Usage of "Active Brains" indicated overall feasibility and satisfactory engagement with the physical activity intervention content (which did not require sustained online engagement), but engagement with online cognitive training was limited. Uptake of the additional brief telephone support appeared to be higher in the "lower cognitive score" trial. Preliminary descriptive trends in the primary outcome data might indicate a protective effect of Active Brains against cognitive decline, but further investigation in fully-powered trials is required to answer this definitively. Discussion: Whilst initial uptake and engagement with the online intervention was modest, it was in line with typical usage of other digital behavior change interventions, and early indications from the descriptive analysis of the primary outcome and behavioral data suggest that further exploration of the potential protective benefits of Active Brains are warranted. The study also identified minor modifications to procedures, particularly to improve online primary-outcome completion. Further investigation of Active Brains will now seek to determine its efficacy in protecting cognitive performance amongst adults aged 60-85 with varied levels of existing cognitive performance.

The Active Brains Digital Intervention to Reduce Cognitive Decline in Older Adults: Protocol for a Feasibility Randomized Controlled Trial.

BACKGROUND: Increasing physical activity, improving diet, and performing brain training exercises are associated with reduced cognitive decline in older adults. OBJECTIVE: In this paper, we describe a feasibility trial of the Active Brains intervention, a web-based digital intervention developed to support older adults to make these 3 healthy behavior changes associated with improved cognitive health. The Active Brains trial is a randomized feasibility trial that will test how accessible, acceptable, and feasible the Active Brains intervention is and the effectiveness of the study procedures that we intend to use in the larger, main trial. METHODS: In the randomized controlled trial (RCT), we use a parallel design. We will be conducting the intervention with 2 populations recruited through GP practices (family practices) in England from 2018 to 2019: older adults with signs of cognitive decline and older adults without any cognitive decline. Trial participants were randomly allocated to 1 of 3 study groups: usual care, the Active Brains intervention, or the Active Brains website plus brief support from a trained coach (over the phone or by email). The main outcomes are performance on cognitive tasks, quality of life (using EuroQol-5D 5 level), Instrumental Activities of Daily Living, and diagnoses of dementia. Secondary outcomes (including depression, enablement, and health care costs) and process measures (including qualitative interviews with participants and supporters) will also be collected. The trial has been approved by the National Health Service Research Ethics Committee (reference 17/SC/0463). RESULTS: Results will be published in peer-reviewed journals, presented at conferences, and shared at public engagement events. Data collection was completed in May 2020, and the results will be reported in 2021. CONCLUSIONS: The findings of this study will help us to identify and make important changes to the website, the support received, or the study procedures before we progress to our main randomized phase III trial. TRIAL REGISTRATION: International Standard Randomized Controlled Trial Number 23758980; http://www.isrctn.com/ISRCTN23758980. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/18929.

Recommendations for starting a grown up congenital heart disease (GUCH) unit.

During the last decades, advances in diagnosis and treatment of congenital heart disease have allowed many individuals to reach adulthood. Due mainly to the great diagnostic diversity and to the co-morbidities usually present in this age group, these patients demand assistance in a multidisciplinary facility if an adequate attention is aimed. In this paper we reviewed, based in the international literature and also on the authors' experience, the structural conditions that should be available for these patients. We highlighted aspects like the facility characteristics, the criteria usually adopted for patient transfer from the paediatric setting, the composition of the medical and para- medical staff taking into account the specific problems, and also the model of outpatient and in-hospital assistance. We also emphasized the importance of patient data storage, the fundamental necessity of institutional support and also the compromise to offer professional training. The crucial relevance of clinical research is also approached, particularly the development of multicenter studies as an appropriate methodology for this heterogeneous patient population.

Effect of atrial flutter on exercise tolerance in patients with grown-up congenital heart (GUCH).

OBJECTIVES: To assess the effect of atrial flutter (AFL) on exercise tolerance in patients with grown-up congenital heart (GUCH), exercise tests with modified Bruce protocol were performed in 20 patients aged 21 to 62 years with GUCH (11 females, 9 males) during symptomatic AFL and again 24 to 48 hours after DC conversion to sinus rhythm (SR). At the same time, cardiac function was assessed by means of transthoracic Doppler echocardiography. RESULTS: Mean exercise duration was significantly less during AFL (6.4 +/- 4.1 min) versus SR (10.9 +/- 3.7 min) (P <.001). Heart rate was faster at rest and peak exercise while in AFL (106 +/- 21 beats/min vs 77 +/- 14 beats/min, P <.001, and 157 +/- 31 beats/min vs 129 +/- 24 beats/min, P <.01, respectively). Systolic blood pressure was lower at peak exercise with AFL (112 +/- 25 mm Hg vs 137 +/- 24 mm Hg, P <.001), as was mean blood pressure increase (5.3 +/- 24.3 mm Hg vs 22.6 +/- 15.8 mm Hg) compared with SR (P <.01). Four of the 6 patients after Fontan surgery had a decrease of 16 mm Hg in systolic blood pressure at peak exercise when in AFL. The reasons for exercise termination during AFL were mainly breathlessness, chest pain, or presyncope, whereas in SR it was caused by fatigue. Echocardiography during AFL showed shorter isovolumic relaxation time (40 +/- 20 ms) compared with SR (50 +/- 20 ms) (P <.05). Ventricular long-axis excursion was reduced (left 1.0 +/- 0.3 cm vs 1.2 +/- 0.4 cm, septal 0.5 +/- 0.2 cm vs 0.7 +/- 0.3 cm, and right 0.7 +/- 0.2 cm vs 0.9 +/- 0.4 cm respectively, P <.001 for all), as were peak pulmonary and aortic flow velocities (85 +/- 30 cm/s vs 105 +/- 50 cm/s, P <.001, and 137 +/- 118 cm/s vs 143 +/- 114 cm/s, P <.02) compared with sinus rhythm. There was a close correlation between exercise duration and blood pressure increase (r = 0.6), left-sided long-axis excursion and blood pressure increase (r = 0.57), and between aortic flow velocity and right-sided long-axis excursion (r = 0.71). CONCLUSIONS: Atrial flutter causes dramatic reduction in exercise tolerance in patients with GUCH, and the combination of fast heart rate and hypotension may contribute to the development of presyncope, particularly in those with Fontan surgery. Marked improvement in effort tolerance and cardiac dynamics occurs after regaining SR. Thus, improving the quality of life in patients with GUCH requires maintaining SR.

Recurrent pneumonia and a normal heart: late complication after repair of hemianomalous pulmonary venous drainage-a cautionary tale.

Hemianomalous pulmonary venous drainage with intact atrial septum is a rare congenital anomaly and reports of its surgical repair and the long-term complications related to the correction are only infrequently encountered in the literature. We report the case of a patient with hemianomalous pulmonary venous drainage and intact atrial septum who underwent surgical repair using a pericardial baffle and creation of an "atrial septal defect" aged 15 years. Dyspnoea and recurrent chest infections started 7 months after surgery when he was seen by a respiratory physician without cardiac followup. He presented again aged 28 years with a recurrent pneumonia investigated over 6 weeks and heart pronounced normal from examination and echocardiography. Correct diagnosis was made in Grown Up Congenital Heart (GUCH) clinic stimulating review of data and catheterisation with pulmonary artery angiography which confirmed it. We feel that this case highlights the importance of specialist care and followup for GUCH patients.

Recommendations for starting a grown up congenital heart disease (GUCH) unit / Recomendações para a implantação de uma Unidade de Cardiopatias Congênitas no Adulto

AbstractDuring the last decades, advances in diagnosis and treatment of congenital heart disease have allowed many individuals to reach adulthood. Due mainly to the great diagnostic diversity and to the co-morbidities usually present in this age group, these patients demand assistance in a multidisciplinary facility if an adequate attention is aimed. In this paper we reviewed, based in the international literature and also on the authors’ experience, the structural conditions that should be available for these patients. We highlighted aspects like the facility characteristics, the criteria usually adopted for patient transfer from the paediatric setting, the composition of the medical and para- medical staff taking into account the specific problems, and also the model of outpatient and in-hospital assistance. We also emphasized the importance of patient data storage, the fundamental necessity of institutional support and also the compromise to offer professional training. The crucial relevance of clinical research is also approached, particularly the development of multicenter studies as an appropriate methodology for this heterogeneous patient population.

ResumoDurante as últimas décadas, os avanços verificados no diagnóstico e tratamento das cardiopatias congênitas têm permitido que muitos indivíduos cheguem à idade adulta. Devido principalmente à grande diversidade diagnóstica e também às comorbidades habitualmente presentes nesse grupo etário, esses pacientes necessitam ser atendidos numa unidade multidisciplinar, se o objetivo for proporcionar uma assistência adequada. Neste trabalho revisamos, com base na experiência dos autores e na literatura internacional, as condições estruturais que devem estar disponíveis para esses pacientes. Procuramos ressaltar aspectos como as características da unidade, o critério usualmente adotado para transferência desses pacientes da unidade pediátrica, a composição das equipes médica e paramédica levando em consideração os problemas específicos dos pacientes e também o modelo de assistência ambulatorial e hospitalar. Enfatizamos, ainda, a importância do armazenamento dos dados dos pacientes, a necessidade fundamental de apoio institucional e a importância de oferecer treinamento profissional. A relevância da pesquisa clínica é também abordada, particularmente a importância da confecção de estudos multicêntricos, como uma metodologia apropriada para essa heterogênea população de pacientes.

Coarctaçäo da aorta: análise pós-operatória da pressäo arterial durante esforço / Aortic coarctation: postoperative analysis of blood pressure during exertion

Quarenta e nove pacientes operados para correçäo de coarctaçäo da aorta foram submetidos a teste cicloergométrico padronizado, tendo os resultados sido comparados com aqueles encontrados em 48 indivíduos sem cardiopatia. A análise comparativa foi feita somente entre os indivíduos que atingiram a freqüência cardíaca submáxima: 38 pacientes (idades 10-63 anos) e 41 controles (idade 8-60 anos). Foram considerados hipertensos durante o esforço os pacientes menores de 15 anos com pressäo sistólica maior de 190 mmHg e os maiores de 15 anos com valores maiores que 210 mmHg na freqüência cardíaca submáxima. Dez (26%) pacientes foram considerados portadores de hipertensäo sistólica de esforço enquanto nenhum paciente apresentou hipertensäo diastólica. Noventa por cento dos pacientes com pressäo normal em repouso teve comportamento normal da pressäo durante o esforço. Oitenta e quatro por cento dos pacientes com pressäo limítrofe em repouso teve hipertensäo sistólica de esforço. Seis indivíduos com pressäo arterial normal no pré-operatório tiveram pressäo normal em repouso e durante o esforço após a operaçäo. Em virtude da elevada freqüência de hipertensäo arterial de esforço após correçäo de coarctaçäo da aorta, recomenda-se que esses pacientes sejam seguidos cuidadosamente e que um teste ergométrico seja empregado rotineiramente para avaliaçäo do comportamento da pressäo arterial. Recomenda-se que esses pacientes evitem atividade física intensa. Um gradiente residual no local da anastamose cirúrgica, que poderia ser exacerbado durante o esforço, parece ser o dado inicial a ser pesquisado para a determinaçäo precisa da causa da hipertensäo arterial nos pacientes operados para correçäo de coarctaçäo da aorta

Coarctaçäo da aorta: análise pós-operatória da pressäo arterial em repouso / Aortic coarctation: postoperative analysis of the blood pressure at rest

Quarenta e nove pacientes tratados cirurgicamente para correçäo de coarctaçäo da aorta torácica foram examinados com o objetivo de avaliar a pressäo arterial em repouso. Foi afastada a presença de uma lesäo renal ou renovascular associada, assim como de recoarctaçäo da aorta. Os resultados revelaram 23% de casos com hipertensäo arterial e 16% com pressäo arterial limítrofe, num período de seguimento médio de 6,8 anos. A média de idade, na época da intervençäo cirúrgica, dos pacientes com pressäo arterial normal foi de 13,5 anos enquanto a dos pacientes com pressäo arterial alterada foi 21 anos (p < 0,05). Relacionando a pressäo arterial atual com idade na época da correçäo cirúrgica, concluiu-se que a intervençäo deve ser realizada antes dos 5 anos de idade. Em 18 pacientes, nos quais foi feita anastomose cirúrgica com "dracon", 6 (33%) tinham arterial arterial alterada. Em 15 pacientes, nos quais foi empregada a anastomose término-terminal, 6 (40%) apresentavam alteraçäo na pressäo arterial, enquanto que entre as 8 pacientes nos quais se usou artéria mamária interna para a anastomose, 5 (62%) tinham arterial alterada. Em vista de alta freqüência de hipertensäo pós-operatória, recomenda-se que esses pacientes sejam cuidadosamente acompanhados com o objetivo de determinar a causa da hipertensäo e de prevenir os riscos potenciais dessa entidade

Qualidade de vida após correção cirúrgica de coarctação da aorta. Análise retrospectiva de um grupo de pacientes seguidos a longo prazo / Quality of Life after Correction of Coarctation of the Aorta. Retrospective Analysis of a Group of Patients Followed in the Long-Term

PURPOSE: To report on the long-term results after operation for coarctation of the aorta. METHODS: One hundred and four patients were studied, divided in four groups (G1, G2, G3 and G4), according to age at operation. Data analysed: reoperation, persistent hypertension, residual lesions, left ventricular function and ability index. RESULTS: Reoperation was frequent, mainly in G1 (60) and G4 (29). Resting hypertension occurred predominantly in cases operated on after the 10th year of life: 28(G3) and 29(G4). Exercise hypertension was found in cases operated on after the 20th year. Residual lesions were frequent: 97, 98, 83and 65(G1 to G4). Individual functional limitation was uncommon. The ability index was normal in the great majority of the patients (94). CONCLUSION: Reoperation is frequent, particularly for recoarctation and aortic stenosis. Rest and/or exercise hypertension is common and related to delayed surgery. Aortic residual lesions are frequent. Physical limitation is uncommon. Postoperative follow-up is essential in order to detect late complications, which, usually, do not limit the individual patient.

Seguimento tardio após correçäo de coarctaçäo da aorta: reintervençäo cirúrgica subsequente / Long-Term Follow-up After Correction of Coarctation of the Aorta. Subsequent Surgical Procedures

PURPOSE--To determine the frequency and main features of subsequent cardiovascular surgery in patients operated on for coarctation of the thoracic aorta. METHODS--One hundred and five patients operated on for coarctation of the aorta with a mean follow-up period of 14 years had their notes analysed. The patients were divided in 4 groups according to age at correction of the coarctation. The incidence of recoarctation repair and other cardiovascular operations were noted. RESULTS--Surgical morbidity was important: 33 per cent . Recoarctation occurred in 14 per cent of the cases, mainly in those who had the coarctation resected during the first year of life. Operation for other cardiovascular defects was necessary in 24.5 per cent of the cases. Among these, correction of left-to-right shunts was done in 50 per cent of the patients who had the coarctation resected in the first year of life. Relief of aortic stenosis was the most frequent procedure (73 per cent ), tends to be more frequent the other is the patient at coarctation repair and more than one procedure may be necessary in some cases. CONCLUSION--Reoperation is frequent in the long term of patients operated on for coarctation of the aorta. The elective coarctation should be repaired after the first year of life hoping to avoid recoarctation. Routine follow-up is advisable for all patients aiming to detect residual left-to-right shunts and left ventricular outflow tract obstruction. Family counseling regarding prognosis after coarctation resection is recommended

Estudo ecocardiográfico bidimensional em fetos de mäes com cardiopatias congênitas / Two-dimensional echocardiographic study in fetus of mothers with congenital heart diseases

Trinta mulheres com idade gestatória de 14 a 34 semanas (média 25 semanas), portadoras de cardiopatias congênitas diversas, foram submetidas a estudo ecocardiográfico para avaliaçäo da anatomia cardíaca fetal. A posiçäo fetal era determinada pela identificaçäo da cabeça, sacro e coluna vertebral. Em seguida, tentava-se reproduzir cortes equivalentes aos comumente usados na ecocardiografia. Os cortes 4-câmaras, eixo menor e eixo maior foram obtidos em 90%, 85% e 50% dos casos, respectivamente. Os estudos foram satisfatórios em 26 fetos (87%). Os resultados inadequados em 4 fetos foram decorrentes da inexperiência inicial e/ou da dificuldade para obter cortes ecocardiográficos apropriados. Os achados permitiram analisar as cavidades cardíacas, septos ventricular e atrial, valvas atrioventriculares e grandes vasos. Informaçöes adicinais, tais como banda moderadora, veias pulmonares e nível de implantaçäo das valvas atrioventriculares foram frequenemente obtidas. A confirmaçäo dos achados ecocardiográficos foi possível em 25 fetos estudados no período pós-natal. A associaçäo dos cortes obtidos permitiu excluir anomalias intracardíacas graves. Conclui-se que é possível a visibilizaçäo da anatomia cardíaca fetal normal e que o método pode ser útil na detecçäo de algumas das cardiopatias congênitas na fase gestatória