New Applications of Cardiopulmonary Exercise Testing and Training in Paediatric Heart Disease

Congenital heart defects (CHD) have an incidence of 4-8/1000 live births and encompass a broad spectrum of disorders. Due to advances in cardiac surgery and cardiology care, most children born with CHD are now surviving into adulthood and there are currently more adults affected than children. Exercise capacity is reduced across the spectrum of patients with CHD, both in natural history and after surgical and interventional treatment. The aim of this project was to better understand exercise limitations and safety/usefulness of training in young patients with heart disease across a broad spectrum of disorders and in particular we focused on left-to-right shunts, systemic right ventricular physiology, univentricular physiology with Fontan palliation and dilated cardiomyopathy. Future perspective include exploring new ways of engaging teenagers with univentricular palliation in systemic and respiratory muscle training. Furthermore, the collaboration with biomedical engineers will allow us to gain in depth understanding of univentricular physiology. Similarities exisit in the physiological changes observed during exercise and pregnancy and the final goal is to implement the model with these variables to achieve better clinical outcome in this growing population of young adults with complex lesions and limited exercise and child-bearing potential. Exercise testing and training are becoming more and more relevant to guide therapy and management but also to assess the ability in daily activities that play an important role in many aspects of life that have not been addressed specifically until now. The increasing data available enable physicians to give adequate counseling regarding vocational or professional choices, suitable leisure activities and family planning according to the levels of activity considered safe and sustainable in the specific physiology. Further studies will warrant deeper understanding of issues that are specific to univentricular physiology and will help us to target interventions to improve quantity and quality of life

Incidental congenital coronary artery vascular fistulas in adults:Evaluation with adenosine-N-13-ammonia PET-CT

AIM To assess the functionality of congenital coronary artery fistulas (CAFs) using adenosine stress N-13-ammonia positron emission tomography computed tomography (PET-CT). METHODS Congenital CAFs were incidentally detected during coronary angiography (CAG) procedures in 11 adult patients (six males and five females) with a mean age of 64.3 years (range 41-81). Patients were collected from three institutes in the Netherlands. The characteristics of the fistulas (origin, pathway and termination), multiplicity of the origins and pathways of the fistulous vessels were assessed by CAG. Five patients underwent adenosine pharmacologic stress N-13-ammonia PET-CT to assess myocardial perfusion and the functional behavior of the fistula. RESULTS Eleven patients with 12 CAFs, 10 unilateral and one bilateral, originating from the left anterior descending coronary artery (n = 8), right coronary artery (n = 2) and circumflex (n = 2). All fistulas were of the vascular type, terminating into either the pulmonary artery (n = 11) or coronary sinus (n = 1). The CAG delineated the characteristics of the fistula (origin, pathway and termination). Multiplicity of the origins and pathways of the fistulous vessels were common in most fistulas (8/12, 67% and 9/12, 75%, respectively). Multiplicity was common among the different fistula components (23/36, 64%). Adenosine pharmacologic stress N-13-ammonia PET-CT revealed normal myocardial perfusion and ejection fraction in all but one patient, who showed a reduced ejection fraction. CONCLUSION PET-CT may be helpful for assessing the functional status of congenital CAFs in selected patients regarding clinical decision-making. Studies with a larger patient series are warranted

Case Reports

Indonesian Society of Interventional Cardiology Annual Meeting 2021   Abstracts: Case Report

Case Reports

Abstracts of the 12th Indonesian Society of Interventional Cardiology Annual Meeting (ISICAM) 2020 27-29 November 202

Percutaneous pulmonary valve implantation in humans - Results in 59 consecutive patients

Background - Right ventricular outflow tract (RVOT) reconstruction with valved conduits in infancy and childhood leads to reintervention for pulmonary regurgitation and stenosis in later life.Methods and Results - Patients with pulmonary regurgitation with or without stenosis after repair of congenital heart disease had percutaneous pulmonary valve implantation (PPVI). Mortality, hemodynamic improvement, freedom from explantation, and subjective and objective changes in exercise tolerance were end points. PPVI was performed successfully in 58 patients, 32 male, with a median age of 16 years and median weight of 56 kg. The majority had a variant of tetralogy of Fallot (n = 36), or transposition of the great arteries, ventricular septal defect with pulmonary stenosis (n = 8). The right ventricular (RV) pressure (64.4 +/- 17.2 to 50.4 +/- 14 mm Hg, P < 0.001), RVOT gradient (33 +/- 24.6 to 19.5 +/- 15.3, P < 0.001), and pulmonary regurgitation ( PR) (grade 2 of greater before, none greater than grade 2 after, P < 0.001) decreased significantly after PPVI. MRI showed significant reduction in PR fraction (21 +/- 13% versus 3 +/- 4%, P < 0.001) and in RV end-diastolic volume (EDV) (94 +/- 28 versus 82 +/- 24 mL (.) beat(-1) (.) m(-2), P < 0.001) and a significant increase in left ventricular EDV ( 64 +/- 12 versus 71 +/- 13 mL (.) beat(-1.) m(-2), P = 0.005) and effective RV stroke volume ( 37 +/- 7 versus 42 +/- 9 mL (.) beat(-1) (.) m(-2), P = 0.006) in 28 patients (age 19 +/- 8 years). A further 16 subjects, on metabolic exercise testing, showed significant improvement in V(O2)max (26 +/- 7 versus 29 +/- 6 mL (.) kg(-1) (.) min(-1), P < 0.001). There was no mortality.Conclusions - PPVI is feasible at low risk, with quantifiable improvement in MRI-defined ventricular parameters and pulmonary regurgitation, and results in subjective and objective improvement in exercise capacity

Platypnoea–orthodeoxia syndrome in a patient on haemodialysis: a case report and scoping review

Introduction: Platypnoea–orthodeoxia syndrome (POS) is a rare condition caused most frequently by an intracardiac right-to-left shunt. Diagnosis requires a high index of suspicion. We report a case of a young man on chronic haemodialysis who developed POS because of superior vena cava obstruction. We also conducted a scoping review of the literature on POS in adult patients. Methods: We followed the PRISMA–ScR guidelines. Studies were eligible for inclusion if they reported on patients with POS in hospitalised adults. We included case reports and case series from 1 January 1949 to 31 May 2023, sought from PubMed (Medline), Web of Science Core Collection, and the Cumulative Index of Nursing and Allied Health Literature. Results: A 29-year-old man undergoing long-term haemodialysis experienced respiratory distress during his dialysis sessions, particularly when seated upright. His oxygen saturation improved to 100% when supine but dropped to 80% when he stood up. Blocking the arteriovenous fistula (AVF) while standing raised his oxygen saturation to 93%. Left heart catheterisation revealed extensive systemic venous collaterals draining into the left atrium via pulmonary veins. A diagnosis of POS from systemic venovenous collateral vessels due to superior vena cava obstruction was made. Because of the patient’s comorbidities and the AVF being his last viable vascular access, no further interventions were pursued. Three hundred and thirty-seven articles (662 patients) were identified in the search of the literature. Six of the patients were on kidney replacement therapy (KRT). The median age was 70 years (IQR 58–79 years). Overall, the most common cause (80%) of POS was an intracardiac right-to-left shunt and patent foramen ovale (PFO) was the most common (74%) primary cause. Hepatopulmonary syndrome (HPS) was the most common extracardiac cause (38%) of POS. Of the six patients on KRT, five had intracardiac causes of POS and one had an extracardiac cause. Most patients (67%) with PFO had percutaneous closure of the shunt. Four of the five patients on KRT with intracardiac shunt had percutaneous or open surgical closure whereas one was managed conservatively. Most of the patients were discharged and had a low mortality rate of only 5%. Conclusions: This case report and literature review describes the causes, treatment, and outcome of POS. Since this is a rare condition, a high index of suspicion is needed for diagnosis. There is a limited number of documented cases in individuals receiving KRT. Although intracardiac shunts were still the most prevalent cause of POS in patients on KRT, SVC obstruction is another aetiology that should be considered. Overall, the prognosis for individuals with POS as reported in the literature was excellent, with a low mortality rate

Long-Term Follow Up of Secundum Atrial Septal Defect Closure with the Amplatzer Septal Occluder

During the past 15 years, closure of a secundum atrial septal defect (ASD) has moved from a surgical to a percutaneous transcatheter approach. Few long-term studies of the efficacy and safety of closure of an ASD by an Amplatzer septal occluder (ASO) exist.To examine the long-term results of secundum ASD closure using the ASO, data on 94 patients who underwent secundum ASD closure with the ASO between 1998 and 2002 were available and reviewed. Data regarding residual shunt, chest pain, palpitations, arrhythmias, headaches, transient ischemic attacks, cerebrovascular accidents, and mortality were collected.Seven (7.4%) subjects had residual shunts immediately following ASO placement. During follow-up, 4 residual shunts closed for a complete closure rate of 97%. Eighteen (19%) patients reported chest pain during the follow-up period. Twenty-three patients (24%) reported palpitations during the follow up period, 7 were documented arrhythmias, including supraventricular tachycardia, atrial fibrillation, and premature ventricular beats. Migraine headaches were new-onset in 4 patients. Migraine cessation occurred in 2 patients after secundum ASD closure. One child died from a cerebral vascular event 18 months following device placement. Only 1 patient developed mild aortic insufficiency.These data indicate that for up to 120 months of patient follow-up, the ASO continues to be a safe device. Residual shunts and arrhythmias have low incidence post-ASO placement. Given the mortality in one high-risk patient, further investigation into anti-platelet therapy after device placement is warranted.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/78631/1/j.1747-0803.2009.00358.x.pd

Surgical Explantation of an Amplatzer Device for Patent Foramen Ovale Closure in a Patient With Nickel Allergy: A Case Report.

Patent foramen ovale (PFO) closure using percutaneous devices, such as the Amplatzer occluder, is a common treatment for patients with a history of cryptogenic stroke or transient ischemic attack (TIA). Although generally well-tolerated, some patients may develop adverse reactions to the device materials, particularly in the presence of a nickel allergy. Symptoms can include chest pain, rashes, and migraines, which may necessitate surgical removal of the device. In such cases, careful surgical planning and execution are essential to ensure successful outcomes and symptom resolution. We present the case of a 39-year-old female with existing PFO closure using an Amplatzer device, who developed severe, persistent atypical chest pain radiating to her upper extremities and occasional migraines 1 month after Amplatzer implantation. Patch testing confirmed a nickel allergy, prompting a referral to our service for surgical explantation of the device. The patient underwent Amplatzer device removal and reconstruction of the interatrial septum via sternotomy. The surgical technique involved meticulous excision of the device while preserving maximal septal tissue, allowing for primary closure of the interatrial septum without the need for a pericardial patch. Intraoperatively, significant scar tissue and inflammation were observed surrounding the device and atrial septal tissue, necessitating careful excision while preserving healthy tissue. The left atrial appendage was oversewn using a polypropylene suture for thromboembolism prophylaxis. Amniotic membrane allograft and autologous platelet-rich plasma were applied to promote wound healing, with specialized suture tapes used for chest closure to avoid using stainless steel sternal wires (the most common alloy, 316 L surgical steel contains around 15% nickel) and minimize the risk of sternal complications. The patient tolerated the procedure well, with complete resolution of symptoms following device removal. At follow-up, she reported improved exercise tolerance, enhanced quality of life, and was able to discontinue her pregabalin medication after 30 months of use (600 mg once daily). She returned to work with light duties 6 weeks postoperatively. This case highlights the importance of preoperative allergy testing to consider nickel allergy in patients before implanting devices such as an Amplatzer, as well as after considering de novo nickel sensitization as a potential cause when such symptoms develop after implantation. This case also highlights the value of surgical intervention in alleviating symptoms and optimizing patient outcomes in cases of device-related complications