The interplay between pressure, flow, and resistance in neonatal pulmonary hypertension.

Pulmonary hypertension, conventionally defined by absolute pulmonary artery pressure, is the result of a range of diagnoses that can result in clinical problems in neonatal practice. Causes include persistent pulmonary hypertension of the newborn, congenital heart disease, and left heart dysfunction, as well as the normally high pulmonary artery resistance in neonates. Elucidating the cause of pulmonary hypertension is vital to guide appropriate management. A first principles approach based on hemodynamic calculations provides a framework for the diagnostic work up and subsequent therapy. Central to this is the equation 'pressure = flow x resistance' and knowledge of factors contributing to flow and resistance and their impact on pulmonary artery pressure. While formal, accurate, calculation of each element is usually not required or deliverable in small infants, clinical and echocardiographic parameters, combined with an understanding of the interplay between pressure, flow, and resistance, significantly improves the assessment and management of neonatal pulmonary hemodynamics.

Consultant staffing in UK congenital cardiac services: a 10-year survey of leavers and joiners.

OBJECTIVES: To report the numbers of consultant congenital cardiac surgeons and cardiologists who have joined and left UK practice over the last 10 years and explore the reasons for leaving. METHODS: Retrospective observational questionnaire study completed between 11 June 2019 and 1 July 2020 by UK level 1 congenital cardiac centres of 10-year consultant staff movement and reasons suggested for leaving UK practice. RESULTS: At survey completion there were 218 (202 whole time equivalent (WTE)) consultant cardiologists and surgeons working within level 1 centres made up of 39 (38 WTE) surgeons, 137 (128.5 WTE) paediatric cardiologists, 42 (35.5 WTE) adult congenital heart disease (ACHD) cardiologists. 161 (74%) consultants joined in the last 10 years of whom 103 (64%) were UK trained. There were 91 leavers giving a staff turnover rate 42% (surgeons 56%, paediatric cardiologists 42%, ACHD cardiologists 29%). Of those, leaving 43% moved to work abroad (surgeons 55%, paediatric cardiologists 40%, ACHD cardiologists 67%). Among the 65 reported reasons for leaving 16 were financial, 9 for work life balance, 6 to working conditions within the National Health Service (NHS) and 12 related to the profession in the UK including six specifically highlighting the national review process. CONCLUSIONS: There has been a high turnover rate of consultant staff within UK congenital cardiac services over the last 10 years with almost half of those leaving moving to work overseas. Financial reasons and pressures relating to working in the NHS or the specialty in the UK were commonly reported themes for leaving. This has major implications for future planning and staff retention within this specialised service.

Outcome following heart transplant assessment in adults with congenital heart disease.

OBJECTIVES: Adults with congenital heart disease (ACHD) are a growing group with end-stage heart failure. We aim to describe the outcomes of ACHD patients undergoing assessment for orthotopic heart transplant (OHT). METHODS: Case notes of consecutive ACHD patients (>16 years) assessed for OHT between 2000 and 2016 at our centre were reviewed. Decision and outcome were reported as of 2017. Data were analysed in three groups: systemic left ventricle (LV), systemic right ventricle (RV) and single ventricle (SV). RESULTS: 196 patients were assessed (31.8 years, 27% LV, 29% RV, 44% SV). 89 (45%) patients were listed for OHT and 67 (34%) were transplanted. 41 (21%) were unsuitable or too high risk and 36 (18%) were too well for listing. Conventional surgery was undertaken in 13 (7%) and ventricular assist device in 17 (9%) with 7 (4%) bridged to candidacy. Survival from assessment was 84.2% at 1 year and 69.7% at 5 years, with no difference between groups. Patients who were considered unsuitable for OHT (HR 11.199, p<0.001) and listed (HR 3.792, p=0.030) were more likely to die than those who were considered too well. Assessments increased over the study period. CONCLUSIONS: The number of ACHD patients assessed for OHT is increasing. A third are transplanted with a small number receiving conventional surgery. Those who are unsuitable have a poor prognosis.

Is alternative cardiac surgery an option in adults with congenital heart disease referred for thoracic organ transplantation?

OBJECTIVES: We analysed the outcomes of adults with congenital heart disease (ACHD) referred for thoracic organ transplantation who underwent non-transplant cardiac surgery as an alternative management option. METHODS: Adult patients with congenital heart disease assessed for heart or heart-lung transplant were identified from the departmental database. A retrospective analysis of the medical records, transplant assessment data and surgical notes was carried out. RESULTS: One hundred and twenty-six patients were assessed between January 2000 and July 2011. Non-transplant cardiac surgery was performed in 14 (11%) patients. There were nine males with a median age of 37 years (range 21-42). The patients can be divided into four subgroups [left-sided lesions (n = 4), right-sided lesions (n = 3), systemic right ventricle (n = 5) and Fontan circulation (n = 2)]. Surgical procedures performed were: relief of systemic obstructive/regurgitant lesions ± endocardial fibroelastosis resection (n = 4, three pulmonary vascular resistance >6 Wood units), correction of right-sided regurgitant/stenotic lesions (n = 3), ventricular assist device for patients with a systemic right ventricle (n = 5) and re-fashioning of the Fontan pathway (n = 2). There were two early (5 and 30 days) and three late deaths (64, 232 and 374 days) with a 1-year mortality of 28%. None of the deaths occurred in patients with a two-ventricle circulation and atrio-ventricular concordance. Nine patients are alive at a median of 433 days (range 204-2456). The New York Heart Association class has improved in all survivors by at least one class at 3 and 6 months (P = 0.004 and 0.003). CONCLUSIONS: Alternative cardiac surgery can be undertaken in selected patients with ACHD referred for cardiopulmonary transplantation with a low mortality in patients with two ventricles and a systemic left ventricle. Ventricular assist devices carry a significant mortality in patients with a systemic right ventricle, although this offers a valuable palliation when there are no other options. The medium and long-term results are awaited.

Tetralogy of fallot with restrictive ventricular septal defect.

We present a case of tetralogy of Fallot with progressive restriction of the ventricular septal defect (VSD) by tricuspid valve tissue. The report includes echocardiographic and catheter images of this rare complication of tetralogy of Fallot. Restriction of the VSD in tetralogy of Fallot is associated with increased surgical-related mortality.