Mitral valve surgery for marantic endocarditis and multiple cerebral embolisation.

Valvular involvement is common in antiphospholipid syndrome (APS) with increased risk of thrombo-embolic events. We report a patient with APS and multiple cerebral infarcts. Echocardiography demonstrated verrucous vegetations of the mitral valve in keeping with marantic endocarditis. The patient underwent successful mitral valve replacement. Post-operative clinical and echocardiographic follow-up showed excellent short term results.

Unexpected sequel of chronic Q-fever endocarditis.

We report a patient with chronic Q-fever endocarditis who was treated with Bentall procedure on two occasions due to persistent endocarditis. A chronic pseudoaneurysm of the aortic root was discovered incidentally by cardiac magnetic resonance and computed tomography eight years post-operatively. Due to chronicity of the pseudoaneurysm and great risk of re-intervention, conservative management was recommended.

Patent ductus arteriosus with Eisenmenger syndrome.

Herein we report a 21 year-old woman with a previously documented patent ductus arteriosus and Eisenmenger physiology. She presented with increasing cyanosis and exercise intolerance which could be explained by a new finding of right to left shunting through an interatrial communication. She was started on Bosentan therapy aiming to reduce the pulmonary pressure with consideration for heart-lung transplantation should any further deterioration occur.

Determining Research Priorities With Teen and Adult Congenital Heart Disease Patients: A Mixed-Methods Study.

Background: Children with congenital heart disease (CHD) are living longer than ever before. This growing cohort of adults with CHD has high medical and psychosocial needs. Also, patients and advocacy groups are justifiably demanding that their voices be heard in all phases of clinical and health services research. Methods: We conducted a first of its kind research priority-setting exercise with teens and adults with moderate-to-complex CHD. Focus groups were held using a fixed, mixed methods, exploratory sequential design. Objectives were to include the patient voice in all phases of the research process, determine the key needs of patients living with CHD, to guide health services research, and identify the "top 10" research priorities of teens and adults living with CHD. Results: Thirty-five patients participated in one of nine 3-hour focus groups where they shared their experiences living with CHD. They expressed a desire for connection with others living with CHD and altruistic motives for participating. Patients with CHD identified a need for information about their disease and prognosis, a need for connection through physical activity and mentorship programmes, and a need for advanced communication with health care teams. Qualitative results correlated well with quantitative ratings to create a patient-derived "top 10" research priorities list. Conclusions: Patients affected by a chronic disease like CHD want to be included in all phases of research. Our research priority-setting exercise in teens and adults with CHD has created a roadmap for clinicians and researchers to investigate issues most important to those living with CHD.

Contexte: Les enfants atteints d'une cardiopathie congénitale vivent plus longtemps que jamais auparavant. Cette cohorte croissante est composée d'adultes atteints de cardiopathie congénitale qui ont des besoins médicaux et psychosociaux importants. Par ailleurs, les patients et les groupes de revendication exigent à juste titre de faire entendre leurs voix lors de toutes les phases des recherches cliniques et de celles sur les services de santé. Méthodologie: Nous avons mené un exercice novateur sur l'établissement des priorités de recherche chez des adolescents et des adultes atteints de cardiopathie congénitale modérée ou complexe. Nous avons organisé des groupes de concertation selon un plan fixe, séquentiel, exploratoire, à méthodes mixtes. Les objectifs étaient de permettre aux patients de se faire entendre lors de toutes les étapes du processus de recherche, de déterminer les besoins clés des patients atteints de cardiopathie congénitale pour orienter les recherches sur les services de santé et d'identifier les 10 principales priorités de recherche chez les adolescents et les adultes atteints de cardiopathie congénitale. Résultats: L'exercice a porté sur 35 patients qui ont participé à l'un des neuf groupes de concertation de trois heures, au cours desquels ils ont fait part de leurs expériences de vie avec une cardiopathie congénitale. Les participants ont indiqué qu'ils souhaitaient former des liens avec d'autres personnes atteintes d'une cardiopathie congénitale et ont donné des motifs altruistes pour participer. Les patients ont reconnu la nécessité d'être informé au sujet de leur maladie et de leur pronostic, de former des liens par le biais de l'activité physique et de programmes de mentorat et de communiquer plus avec les équipes soignantes. Il existe une corrélation étroite entre les résultats qualitatifs et les évaluations quantitatives, ce qui a permis d'établir une liste des 10 principales priorités de recherche des patients. Conclusions: Les patients qui sont aux prises avec une maladie chronique comme la cardiopathie congénitale souhaitent être inclus dans toutes les phases des travaux de recherche. Par ailleurs, l'exercice sur l'établissement des priorités de recherche que nous avons effectué chez les adolescents et les adultes atteints d'une cardiopathie congénitale a permis de créer une feuille de route pour les cliniciens et les chercheurs. En effet, ce plan leur permettra d'étudier les questions les plus importantes pour les personnes qui vivent avec une cardiopathie congénitale.

Adult congenital heart disease nurse coordination: Essential skills and role in optimizing team-based care a position statement from the International Society for Adult Congenital Heart Disease (ISACHD).

BACKGROUND: Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with congenital heart disease (CHD) worldwide. Among ISACHD's objectives is to "promote a holistic team-based approach to the care of the adult with CHD that is comprehensive, patient-centered, and interdisciplinary" (http://www.isachd.org). This emphasis on team-based care reflects the fact that adults with CHD constitute a heterogeneous population with a wide spectrum of disease complexity, frequent association with other organ involvement, and varied co-morbidities and psychosocial issues. METHODS: Recognizing the vital role of the adult CHD (ACHD) nurse coordinator (ACHD-NC) in optimizing team-based care, ISACHD established a task force to elucidate and provide guidance on the roles and responsibilities of the ACHD-NC. Acknowledging that nursing roles can vary widely from region to region based on factors such as credentials, scopes of practice, regulations, and local culture and tradition, an international panel was assembled with experts from North America, Europe, East Asia, and Oceania. The writing committee was tasked with reviewing key aspects of the ACHD-NC's role in team-based ACHD care. RESULTS/CONCLUSION: The resulting ISACHD position statement addresses the ACHD-NC's role and skills required in organizing, coordinating, and facilitating the care of adults with CHD, holistic assessment of the ACHD patient, patient education and counseling, and support for self-care management and self-advocacy.

Non-invasive Investigations for the Diagnosis of Fontan-Associated Liver Disease in Pediatric and Adult Fontan Patients.

Fontan-associated liver disease (FALD) is a serious complication related to the chronically elevated venous pressure and low cardiac output of this abnormal circulation. However, diagnostic markers for this condition are limited. We hypothesized that specific tests for fibrosis developed for other chronic liver diseases would identify a higher prevalence of FALD than ultrasound and standard laboratory tests and that identified abnormalities would correlate with time post-Fontan. In this cross-sectional study, we assessed 19 children (average age 8.4 ± 4.3 and 5.4 ± 4.1 years post-Fontan) and 8 adults (average age 31.5 ± 8.9 and 21.1 ± 4 years post-Fontan) using standard serum laboratory investigations assessing hepatic integrity and function, the FibroTest, liver ultrasound, and transient elastography (FibroScan). In adult Fontan patients, hemoglobin, C-reactive protein, and gamma-glutamyl transpeptidase were significantly increased, and white blood cell and platelet counts were significantly decreased in comparison to the pediatric cohort. International normalized ratio was mildly elevated in both children and adults. FibroTest results were suggestive of fibrosis regardless of time post-Fontan. FibroScan measurements were significantly correlated with time post-Fontan, but the incidence of ultrasound-detected liver abnormalities was variable. No cases of hepatocellular carcinoma were identified. Abnormalities suggestive of FALD occur in both children and adults post-Fontan. Select laboratory tests, and possibly ultrasound and FibroScan in some patients, appear to have the most promise for the non-invasive detection of FALD.

Contraceptive Practices of Women With Complex Congenital Heart Disease.

Understanding the contraceptive practices of women with complex congenital heart disease (CHD) and providing them individualized contraception counseling may prevent adverse events and unplanned high-risk pregnancies. Given this, we sought to examine the contraceptive practices in women with CHD, describe adverse events associated with contraceptive use, and describe the provision of contraception counseling. Women >18 years were recruited from 2011 to 2014 from 9 adult CHD (ACHD) centers throughout North America. Subjects completed a 48-item questionnaire regarding contraceptive use and perceptions of contraception counseling, and a medical record review was performed. Of 505 subjects, median age was 33 (interquartile range 26 to 44) and 81% had CHD of moderate or great complexity. The majority (86%, 435 of 505) of the cohort had used contraception. The types included barrier methods (87%), oral contraception (OC) 84%, intrauterine device (18%), Depo-Provera (15%), vaginal ring (7%), patch (6%), hormonal implant (2%), Plan B (19%), and sterilization (16%). Overall OC use was not significantly different by CHD complexity. Women with CHD of great complexity were more likely to report a thrombotic event while taking OC than those with less complex CHD (9% vs 1%, p = 0.003). Contraception counseling by the ACHD team was noted by 43% of subjects. Unplanned pregnancy was reported by 25% with no statistical difference by CHD complexity. In conclusion, contraceptive practices of women with complex CHD are highly variable, and the prevalence of blood clots while taking OC is not insignificant while provision of contraception counseling by ACHD providers appears lacking.

Looking beyond the biophysical realm to optimize health: results of a survey of psychological well-being in adults with congenital cardiac disease.

BACKGROUND: The purpose was to examine the relationship between the complexity of structural heart disease and psychological well-being in adults with congenital cardiac disease. METHODS: A total of 380 patients registered at the Adult Congenital Heart Clinic in Calgary, Alberta, Canada were surveyed. The patients were mailed a socio-demographic questionnaire and an instrument to measure psychological well-being. The instrument of psychological well-being measures six dimensions: positive relations with others, autonomy, environmental mastery, personal growth, purpose in life, and self-acceptance. Of the 380 patients surveyed, 85 of 205 patients with simple structural malformation responded, giving a response rate of 41%, and 78 of 175 patients with complex malformations, thus giving a response of 45%. RESULTS: There was no statistically significant difference in the mean scores of each of the six dimensions of psychological well-being between those patients with simple and complex malformations. Two-way analysis of variance, and multivariate analysis of variance, found significant differences in mean scores for dimensions when other socio-demographic variables were included in the analysis. For example, significantly higher mean scores for the dimension of purpose in life was found in patients who obtained a higher level of education (p = 0.009), and in patients who were employed (p < 0.001). We present the socio-demographic variables that statistically impact the mean scores for the dimension of psychological well-being. CONCLUSION: Psychological well-being is not affected by the complexity of the structural congenital cardiac disease. Certain socio-demographic variables that impact psychological well-being, nonetheless, must be considered when developing multidisciplinary programmes to care for young adults with congenitally malformed hearts.