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Incidence and outcomes of prosthetic valve endocarditis in adults with tetralogy of Fallot
- Alexander C. Egbe, Srikanth Kothapalli, William R. Miranda, Raja Jadav, Keerthana Banala, Rahul Vojjini, Faizan Faizee, Fouad Khalil, Maria Najam, Mounika Angirekula, Daniel C. Desimone, Heidi M. Connolly
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- Journal:
- Cardiology in the Young / Volume 30 / Issue 1 / January 2020
- Published online by Cambridge University Press:
- 08 January 2020, pp. 19-23
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Background:
The risk of endocarditis varies with CHD complexity and the presence of prosthetic valves. The purpose of the study was therefore to describe incidence and outcomes of prosthetic valve endocarditis in adults with repair tetralogy of Fallot.
Methods:Retrospective review of adult tetralogy of Fallot patients who underwent prosthetic valve implantation, 1990–2017. We defined prosthetic valve endocarditis-related complications as prosthetic valve dysfunction, perivalvular extension of infection such abscess/aneurysm/fistula, heart block, pulmonary/systemic embolic events, recurrent endocarditis, and death due to sepsis.
Results:A total of 338 patients (age: 37 ± 15 years) received 352 prosthetic valves (pulmonary [n = 308, 88%], tricuspid [n = 13, 4%], mitral [n = 9, 3%], and aortic position [n = 22, 6%]). The annual incidence of prosthetic valve endocarditis was 0.4%. There were 12 prosthetic valve endocarditis-related complications in six patients, and these complications were prosthetic valve dysfunction (n = 4), systemic/pulmonary embolic events (n = 2), heart block (n = 1), aortic root abscess (n = 1), recurrent endocarditis (n = 2), and death due to sepsis (n = 1). Three (50%) patients required surgery at 2 days, 6 weeks, and 23 weeks from the time of prosthetic valve endocarditis diagnosis. Altogether three of the six (50%) patients died, and one of these deaths was due to sepsis.
Conclusions:The incidence, complication rate, and outcomes of prosthetic valve endocarditis in tetralogy of Fallot patients underscore some of the risks of having a prosthetic valve. It is important to educate the patients on the need for early presentation if they develop systemic symptoms, have a high index of suspicion for prosthetic valve endocarditis, and adopt a multi-disciplinary care approach in this high-risk population.
Caring for adults with congenital cardiac disease: successes and challenges for 2007 and beyond
- Joseph A. Dearani, Heidi M. Connolly, Richard Martinez, Hector Fontanet, Gary D. Webb
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- Journal:
- Cardiology in the Young / Volume 17 / Issue S4 / September 2007
- Published online by Cambridge University Press:
- 26 November 2007, pp. 87-96
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Patients with congenital cardiac disease require lifelong medical care. Current challenges that face practitioners who care for adults with congenital heart disease include identifying the best location for procedures, which could be a children’s hospital, an adult hospital, or a tertiary care facility; providing appropriate antenatal management of pregnant women with congenitally malformed hearts, and continuing this care in the peripartum period; and securing the infrastructure and expertise of the non-cardiac subspecialties, such as nephrology, hepatology, pulmonary medicine, and haematology. The objectives of this review are to outline the common problems that confront this population of patients and the medical community, to identify challenges encountered in establishing a programme for care of adults with congenitally malformed hearts, and to review the spectrum of disease and operations that have been identified in a high volume tertiary care centre for adult patients with congenital cardiac disease. Three chosen examples of the fundamental problems facing the practitioner and patient in the United States of America in 2007 are the neglected patient with congenital cardiac disease, weak infrastructure for adults with congenital cardiac disease, and family planning and management of pregnancy for patients with congenital cardiac disease.
Patients with adult congenital cardiac disease often do not receive appropriate surveillance. Three fundamental reasons for this problem are, first, that most adults with congenitally malformed hearts have been lost to follow-up by specialists, and are either receiving community care or no care at all. Second, patients and their families have not been educated about their malformed hearts, what to expect, and how to protect their interests most effectively. Third, adult physicians have not been educated about the complexity of the adult with a congenitally malformed heart. This combination can be fatal for adults with complications related to their congenitally malformed heart, or its prior treatment. Two solutions would improve surveillance and care for the next generation of patients coming out of the care of paediatric cardiologists. The first would be to educate patients and their families during childhood and adolescence. They would learn the names of the diagnoses and treatments, the problems they need to anticipate and avoid, the importance of expert surveillance, career and family planning information, and appropriate self-management. The second solution would be to encourage an orderly transfer of patients from paediatric to adult practice, usually at about 18 years of age, and at the time of graduation from high school.
Clinics for adults with congenital cardiac disease depend upon multidisciplinary collaboration with specialties in areas such as congenital cardiac imaging, diagnostic and interventional catheterization, congenital cardiac surgery and anaesthesia, heart failure, transplantation, electrophysiology, reproductive and high risk pregnancy services, genetics, pulmonary hypertension, hepatology, nephrology, haematology, and others. None of these services are easily available “off the rack”, although with time, experience, and determination, these services can develop very well. Facilities with experienced personnel to provide competent care for adults with congenital cardiac disease are becoming increasingly available. Parents and patients should learn that these facilities exist, and be directed to one by their paediatric caregivers when the time comes for transition to adult care.
With the steady increase in the number of adults with congenital heart disease, an ever increasing number of women with such disease are becoming pregnant. Services are not widely available to assess competently and plan a pregnancy for those with more complex disease. It is essential to have a close interplay between the obstetrician, the adult congenital cardiologist, the fetal medicine perinatologist, and neonatologist.
In both a community based programme and a tertiary care centre, the nuances and complexities of congenital cardiac anatomy, coupled with the high probability of previous operation during childhood, makes the trained congenital cardiothoracic surgeon best suited to deal with the surgical needs of this growing population. It is clear that the majority of adults with congenital heart disease are not “cured”, but require lifelong comprehensive care from specialists who have expertise in this complex arena. There is a growing cadre of healthcare professionals dedicated to improving the care of these patients. More information has become available about their care, and will be improved upon in the next decade. With the support of the general paediatric and paediatric cardiologic communities, and of the Adult Congenital Heart Association, and with the persistence of the providers of care for adults with congenital cardiac disease currently staffing clinics, the care of these patients should become more secure in the next decade as we mature our capabilities.
Clinical features and surgical outcome in 25 patients with fenestrations of the coronary sinus
- Christine H. Attenhofer Jost, Heidi M. Connolly, Gordon K. Danielson, Joseph A. Dearani, Carole A. Warnes, A. Jamil Tajik
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- Journal:
- Cardiology in the Young / Volume 17 / Issue 6 / December 2007
- Published online by Cambridge University Press:
- 24 October 2007, pp. 592-600
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Objective
To analyze symptoms, associated anomalies, diagnostic approach, and surgical procedures in patients with partial fenestrations of the coronary sinus, a rare congenital disorder.
BackgroundPartial fenestrations of the walls that usually separate the coronary sinus from the left atrium, also known as partial unroofing of the coronary sinus, may result in increased flow of blood to the lungs, cyanosis, or bidirectional shunting. The diagnosis is important, but difficult to confirm.
MethodsWe reviewed retrospectively the clinical, echocardiographic, operative, and follow-up data on 25 patients with partial fenestration of the coronary sinus. The patients had a median age of 8 years, and underwent cardiovascular surgery at Mayo Clinic between 1958 and 2003.
ResultsThe initial diagnosis of a fenestration of the coronary sinus was made by the surgeon at repair of other congenital cardiac anomalies, by cardiac catheterization, or by echocardiography. In 14 patients, fenestration was missed during previous cardiovascular surgery. The most common associated cardiac lesions were atrial septal defects within the oval fossa, persistent left or right superior caval veins, and pulmonary or tricuspid atresia. In 7 patients, the symptoms were at least partially attributable to the fenestration, and included dyspnea, cerebral abscess, transient ischaemic attacks, and cyanosis. The fenestration was addressed surgically in 23 patients, and consisted of closure of the mouth of the coronary sinus, creation of an intra-atrial baffle, or direct repair of the fenestration. Death occurred in 1 patient due to complications unrelated to the repair. In the 24 early survivors, who have been followed up for a median of 85 months, 1 patient has died after a third reoperation for complex congenital cardiac disease.
ConclusionsFenestrations of the coronary sinus are often missed in the preoperative evaluation of congenitally malformed hearts. When associated with right heart hypoplasia, atrial septal defect, or persistent superior caval vein, fenestrations of the coronary sinus should be considered as a possible additional finding. Once the diagnosis has been made, repair is straightforward.