Caesarean delivery in a parturient with a femoro-femoral crossover graft and congenital aortic stenosis repaired by the Ross procedure

We report a case of a patient with congenital aortic stenosis previously repaired using the Ross procedure, who presented to our unit for urgent caesarean delivery. Management was complicated by moderate residual cardiac disease and the presence of a suprapubic femoro-femoral crossover graft. Following application of five-lead electrocardiogram and invasive blood pressure monitoring, anaesthesia was induced via combined spinal-epidural with epidural volume extension. A high transverse surgical approach avoided the course of the vascular graft, while further precautions included the immediate availability of vascular surgeons and cell salvage. Our anaesthetic technique was tailored to minimise disruption to cardiovascular function, and in particular to limit regurgitant flow across the pulmonary valve. This case highlights the value of early identification of high-risk parturients and multidisciplinary involvement at delivery. Risk stratification in the patient with grown-up congenital heart disease is based upon timely evaluation of the underlying congenital pathology, surgical history and subsequent functional status.     

Precordial abscess inducing chest pain 20 years after surgical repair of a pentalogy of fallot

A 25-year-old male asylum-seeker presented with chest pain, exertional dyspnea, and orthopnea 20 years after the surgical repair of a pentalogy of Fallot. An extracardiac mass compressing the right ventricle was subsequently detected and surgical decompression was performed to relieve the resulting right intraventricular hypertension. At operation, the mass proved to be a coagulase-negative, staphylococcal abscess. In addition, the removal of the mass unmasked a previously nonrecognized pulmonary outflow stenosis that required balloon dilatation and beta-blocker therapy. While infections are known to occur after sternotomy, the formation of an abscess in the anterior mediastinum several years after the intervention appears to be exceptional; this diagnosis came to mind only after the more common complications had been considered, e.g., pseudoaneurysm or pericardial hematoma. To our knowledge, this is the first report of an abscess in the anterior mediastinum that had probably formed over many years following a sternotomy, compressed the right ventricle and masked a pulmonary stenosis.     

Update in Adult Congenital Cardiac Surgery

The number of grown-up patients with congenital heart disease (GUCH) is constantly increasing and will equal the number of children requiring surgery for congenital heart disease (CHD). Specialized centers dealing with the medical and paramedical problems of these patients are required. GUCH patients can be divided into the following groups: (1) patients with minor cardiac malformations presenting at adult age for first treatment; (2) patients presenting for correction as adults because they are either naturally balanced or were surgically palliated; (3) patients presenting for expected reoperations after correction in childhood; (4) patients requiring repair of residual defects after correction; (5) patients developing heart failure after correction or palliation of CHD requiring thoracic transplantation; and (6) patients developing acquired heart disease in addition to CHD. Special aspects of malformations frequently occurring in GUCH patients are discussed in detail. Acquired heart disease in this patient population is expected to increase in the coming decades as survivors of CHD grow old.     

Young adults experiences of living with congenital heart disease

BACKGROUND: Advances in care have improved the longevity and quality of life for children with congenital heart disease; however, many of them need lifelong highly qualified specialist care. The cardiac lesion involved may not always be the patient's main problem; issues related to quality of life may dominate. AIM: To explore and gain a deeper understanding of young adults experiences of living with congenital heart disease in order to enhance the quality of care provided by the health care system. METHOD: Transcribed in-depth interviews were analysed using a phenomenological-hermeneutic method. Six adults with congenital heart disease, aged 22-39 years old, were included in the study. RESULTS: Analyses yielded two themes, having the disease and living with illness, both originating from the main theme of ambivalence. The interviewees were ambivalent in how they viewed themselves, how they faced their daily life and how they dealt with their encounters with the health care system. They had to strike a balance between being different and not being different; being sick and being healthy; revealing their congenital heart disease or hiding it and living with a hidden handicap. They also had to cope with the disease and with the health care system. CONCLUSION: Young adults with congenital heart disease are ambivalent. They have a strong wish to be healthy and they might hide their symptoms from the healthcare personnel and sometimes even from themselves. A psychosocial preparedness when meeting these patients is necessary.     

The impact of interventional cardiology for the management of adults with congenital heart defects.

The objective of this study is to assess the impact of interventional cardiology procedures for the management of ACHD. The interventional approach to the management of CHD in the adult population is becoming increasingly recognized as the preferred treatment option for a wide number of congenital cardiac conditions. The files of all consecutive patients over 18 years of age who were hospitalized in our department from January 2000 to December 2004 were reviewed. Over the study period, 1,115 ACHD (583 women; mean age 41 +/- 13.8, years, range 18-72 years) were hospitalized in our department; 752 patients underwent cardiac catheterization and 82.4% of them had an interventional procedure carried out. ASD (329/620) and PFO (159/620) closure account for 78% of all the procedures carried out, with a 2.7% of major complications incidence (all of them closing ASDs). Other procedures such as stenting aortic coarctation (40/620), ventricular septal defect closure (33/620), patent ductus arteriosus embolization (30/620), pulmonary valvuloplasty (12/620), stenting pulmonary artery branches (8/620), etc (5/620) were carried out. The most important complication was one death, which occurred in the case of a 22-year-old woman after stent implantation for a recurrent aortic coarctation. A trivial residual shunt was detected in only 5% of the patients who had a 6-month follow-up after VSD closure; no residual shunt was found after PDA embolization during the 12-month follow-up. In conclusion, we believe that the interventional approach is a safe and successful treatment option for a wide number of congenital cardiac conditions. The increasing use of catheter interventions for these patients will be responsible for an increase of complex cases in surgery.     

Recommendations to organize care for adults with congenital heart disease in the Czech Republic

This paper was formed by the Expert committee for congenital heart disease (CHD) in adults – a division of Czech Society of Cardiology (ČKS). It was designed as an appendix to National cardiovascular programme ČKS created in 2013 and was based on Recommendations for organization of care for adults with congenital heart disease and for training in the subspecialty of ‘Grown-up Congenital Heart Disease’ in Europe: a position paper of the Working Group on Grown-up Congenital Heart Disease of the European Society of Cardiology created in 2014 [1].Aims of this paper are: To optimize medical care in all its aspects for adults with CHD in the Czech Republic, to facilitate easy transition between paediatric and adult medical care, to enable research in the field in order to create evidence based care, to support training of regional cardiologists and other specialists who are involved in monitoring adult patients with CHD, to help with communication with national institutions, to provide information for other medical workers and patients, to consolidate resources.     

Totally peripheral approach for ICD lead vegetation removal in a GUCH patient

AngioVac system (AngioDynamics) has already proved to be a useful tool in the treatment of thrombotic and endocarditic formations concerning the venous district. Herein, the AngioVac aspiration system combined with the bidirectional rotational Evolution mechanical sheath lead extraction was used for an effective and safety cardiac-device-related-infective-endocarditis removal in a grown-up congenital heart disease patient through a totally peripheral approach.     

Small unrepaired atrial septal defects display impaired exercise capacity compared with healthy peers

Objective: Adult patients with small, unrepaired atrial septal defects have an in‐creased risk of pneumonia, atrial fibrillation, and stroke. Furthermore, they have higher late mortality than the background population. The functional capacity is un‐known in these patients. Therefore, our objective was to determine exercise capacity in adult patients diagnosed with an unrepaired atrial septal defect compared to healthy controls.Design: A cross‐sectional study.Patients: Adult patients with small, unrepaired atrial septal defects, aged 18‐65, di‐agnosed between 1953 and 2011.Interventions: Cardiopulmonary exercise test was performed using an incremental bicycle test and gas exchange was measured using breath‐by‐breath technique.Outcome measures: Primary outcome was peak oxygen uptake, secondary outcome was maximal workload and ventilatory anaerobic threshold.Results: We included 32 patients previously diagnosed with a small, unrepaired atrial septal defect and 16 healthy, age‐ and gender‐matched controls (age 36.3 ± 13 years). Patients were divided into two groups based on whether the atrial septal defect was open (age 36.3 ± 11 years) or spontaneously closed (age 36.8 ± 14 years) since time of diagnosis. No differences in demographic characteristics or weekly exercise levels were found. Both patient groups reached lower peak oxygen uptake (open: 31.7 ± 11 mL/kg/min; spontaneously closed: 29.7 ± 6.9 mL/kg/min) compared with controls (42.6 ± 6.1 mL/kg/min; P = .0001). Workload (open: 2.6 ± 1.0 watt/kg; spontaneously closed: 2.5 ± 0.6 watt/kg) and aerobic capacity (open: 21.4 ± 8.7 mL/kg/min; spontaneously closed: 22.5 ± 6.5 mL/kg/min) was also poorer in patients compared to controls (workload: 3.5 ± 0.5 watt/kg; P = .0006, aerobic capacity: 31.3 ± 6.8 mL/kg/min; P = .0007).Conclusion: Adult patients with a diagnosis of small, unrepaired atrial septal defect have significantly impaired exercise capacity when compared to healthy controls. The impairment was present even if, by the time of assessment, the defect had closed spontaneously. The pathophysiological mechanisms behind the impaired exercise ca‐pacity demonstrated in these patients remain unexplained and will be a target for future work.     

Measures of exercise capacity in adults with congenital heart disease

Background

Exercise capacity in grown-ups with congenital heart disease (GUCH) is mostly reported by peak oxygen consumption (peak VO₂). Our aim was to evaluate the maximal character of exercise tests, and to investigate submaximal measures of exercise capacity.

Methods

Adults with Coarctation of the Aorta (COA, n = 155), Tetralogy of Fallot (TOF, n = 98), dextro-Transposition of the Great Arteries (dTGA, n = 68) and Univentricular Heart (UVH, n = 10), and 122 healthy adults performed cardiopulmonary exercise testing until exhaustion. Gas exchange was measured breath by breath. The maximal performance of the test was evaluated by respiratory exchange ratio (RER), ventilatory equivalent for oxygen and Borg scale. Oxygen uptake efficiency slope (OUES), VE/VCO₂ slope and VO₂/WR slope were calculated and ventilatory anaerobic threshold (VAT) was defined. Correlations of these measures with peak VO₂ were calculated.

Results

GUCH showed significantly lower peak VO₂ than controls (p < 0.001), declining from 80% in COA, 74% in TOF, 64% in dTGA, to 55% in UVH. Compared to suggested criteria, mean peak RER and median Borg scale indicated a maximal effort in GUCH, however these results were significantly lower than controls (p < 0.05). OUES, VO₂/WR slope and VAT were significantly lower in patients compared to controls. OUES (r = 0.853) and VAT (r = 0.840) correlated best with peak VO₂; VO₂/WR slope (r = 0.551) and VE/VCO₂ slope (r = −0.421) correlated to a lesser degree (p < 0.001).

Conclusion

The investigated GUCH show reduced exercise tolerance compared to controls, related to the underlying heart defect. Different expressions of exercise tolerance clearly reveal the same differences in exercise capacity across groups of GUCH.