Maryanne Caruana

Pulmonary hypertension and pregnancy outcomes: data from the Registry Of Pregnancy and Cardiac Disease (ROPAC) of the European Society of Cardiology.

To describe the outcomes of pregnancy in women with pulmonary hypertension.

Patient-reported outcomes in adults with congenital heart disease: Inter-country variation, standard of living and healthcare system factors

AIMS Geographical differences in patient-reported outcomes (PROs) of adults with congenital heart disease (ConHD) have been observed, but are poorly understood. We aimed to: (1) investigate inter-country variation in PROs in adults with ConHD; (2) identify patient-related predictors of PROs; and (3) explore standard of living and healthcare system characteristics as predictors of PROs. METHODS AND RESULTS Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study (APPROACH-IS) was a cross-sectional, observational study, in which 4028 patients from 15 countries in 5 continents were enrolled. Self-report questionnaires were administered: patient-reported health (12-item Short Form Health Survey; EuroQOL-5D Visual Analog Scale); psychological functioning (Hospital Anxiety and Depression Scale); health behaviors (Health Behavior Scale-Congenital Heart Disease) and quality of life (Linear Analog Scale for quality of life; Satisfaction With Life Scale). A composite PRO score was calculated. Standard of living was expressed as Gross Domestic Product per capita and Human Development Index. Healthcare systems were operationalized as the total health expenditure per capita and the overall health system performance. Substantial inter-country variation in PROs was observed, with Switzerland having the highest composite PRO score (81.0) and India the lowest (71.3). Functional class, age, and unemployment status were patient-related factors that independently and consistently predicted PROs. Standard of living and healthcare system characteristics predicted PROs above and beyond patient characteristics. CONCLUSIONS This international collaboration allowed us to determine that PROs in ConHD vary as a function of patient-related factors as well as the countries in which patients live.

Pregnancy in atriopulmonary connection and total cavopulmonary connection - a comparison of two cases.

S INCE FONTAN AND BAUDET DESCRIBED THE radical palliation for patients with tricuspid atresia, there have been many modifications to the principle and extension to other conditions where biventricular repair is not possible. There are reports of successful pregnancy in women having had Fontan-type surgery but the different techniques used are not clearly separated. This article reports two pregnancies with a Fontan-type circulation from our centre, one in a patient with total cavopulmonary connection and the other with atriopulmonary connection, comparing and contrasting the complications.

Regional variation in quality of life in patients with a Fontan circulation: A multinational perspective

Background Impaired quality of life (QOL) is associated with congenital heart disease (CHD) and country of residence; however, few studies have compared QOL in patients with differing complexities of CHD across regional populations. The current study examined regional variation in QOL outcomes in a large multinational sample of patients with a Fontan relative to patients with atrial septal defects (ASDs) and ventricular septal defects (VSDs). Methods From the Assessment of Patterns of Patient‐Reported Outcomes in Adults with Congenital Heart disease—International Study (APPROACH‐IS), 405 patients (163 Fontan and 242 ASD/VSD) across Asia, Europe, and North America provided consent for access to their medical records and completed a survey evaluating QOL (0 to 100 linear analog scale). Primary CHD diagnosis, disease complexity, surgical history, and documented history of mood and anxiety disorders were recorded. Differences in QOL, medical complications, and mood and anxiety disorders between Fontan and ASD/VSD patients, and across geographic regions, were examined using analysis of covariance. Hierarchical regression analyses were conducted to identify variables associated with the QOL ratings. Results Patients with a Fontan reported significantly lower QOL, and greater medical complications and mood and anxiety disorders relative to patients with ASD/VSD. Inpatient cardiac admissions, mood disorders, and anxiety disorders were associated with lower QOL among patients with a Fontan, and mood disorders were associated with lower QOL among patients with ASD/VSD. Regional differences for QOL were not observed in patients with a Fontan; however, significant differences were identified in patients with ASD/VSD. Conclusions Regional variation of QOL is commonplace in adults with CHD; however, it appears affected by greater disease burden. Among patients with a Fontan, regional variation of QOL is lost. Specific attempts to screen for QOL and mood and anxiety disorders among CHD patients may improve the care of patients with the greatest disease burden.

Aneurysmal dilatation of the aortic sinuses of Valsalva — beyond Marfan syndrome: a single centre experience and review of the literature

Aneurysmal dilatation of the aortic sinuses of Valsalva has been most extensively documented in the setting of aortopathies, particularly Marfan syndrome. On the other hand, there is limited data in the literature about congenital sinus of Valsalva aneurysms outside this context. For the purpose of this review, we carried out a literature search on aneurysmal dilatation of the sinuses of Valsalva in Marfan syndrome, and compared this with congenital sinus of Valsalva aneurysms, also including data from a case series from our institution. In conclusion, there are differences in management of aortic dilatation in Marfan syndrome and congenital sinus of Valsalva aneurysms. Though less well-recognised, congenital aneurysms are often associated with significant morbidity and mortality and timely intervention is necessary.

Lifestyle Habits among Adult Congenital Heart Disease Patients in Malta.

OBJECTIVE Adult congenital heart disease patients are prone to health risk behaviors like their peers. Such behaviors can have serious implications, particularly in patients with complex congenital lesions. We investigated patterns in tobacco smoking, alcohol consumption, substance misuse, dental reviews, and physical activity in Maltese patients. DESIGN A questionnaire modeled on the 2008 European Health Interview Survey was offered to consecutive adult congenital heart disease outpatients, aged 16 years and over, between May 2013 and May 2014. Foreigners and patients with learning difficulties or cognitive impairment were excluded. Data on these habits was compared with that from 372 age-matched and sex-matched 2008 survey responders (general population cohort). RESULTS There were 125 patient responders (65 males; mean age 30.64, SD 12.80 years). Adult congenital heart disease patients smoked less (patients 18.4%, general 32.0% P = .004) and had more regular dental reviews (patients 60.9%, general 45.2% P = .003) but were less physically active than the general population cohort (patients 30.6%, general 62.9% P < .001). The difference in smoking was only significant in patients born after the mid-1970s (younger) and females, while dental reviews were only better in younger and male patients. Male patients consumed alcohol more frequently (38.1%) than general population males (23.3%, P = .02). In the patient cohort, smoking was more likely with milder congenital lesions (OR 4.689, P = .007), substance misuse more likely in males (OR 5.703, P = .036) and with milder lesions (OR 5.405, P = .030) and alcohol consumption more likely in males (OR 11.697, P < .001). CONCLUSIONS Although there is an overall lower uptake of some unhealthy habits by Maltese adult congenital heart disease patients, this is not consistent between sexes or age groups. Male and older patients and those with milder lesions might need more reinforcement of the advice given regarding lifestyle habits. All patients need targeted exercise prescription to ensure better physical activity levels.

Sinus of Valsalva aneurysms in non-marfan patients: an underestimated entity? A case series.

Aims: Sinus of Valsalva aneurysms are rare anomalies. Aneurysmal dilatation of the sinuses of Valsalva in Marfan syndrome has been extensively studied. The aim of this study was to investigate clinical manifestation, diagnosis, histopathological findings and management of sinus of Valsalva aneurysms in non-Marfan patients. Study Design: Retrospective analysis, case-series. Methodology: A search of digital echocardiographic recordings, histopathology and electronic patient databases in our institution for the period 2004 – 2012 was performed. All patients with asymmetrical dilatation of at least one coronary sinus of Valsalva at postmortem and ruptured (or dissected) aneurysms on echocardiogram were included. Patients with Marfan syndrome and those with non-ruptured sinus of Valsalva aneurysms on echocardiogram were excluded. Results: A total of 12 patients (7 males; mean age 36.1 years) were studied. The right coronary sinus was the most commonly affected (9/12). Involvement of more than 1 sinus was seen in 3/12 cases. Four patients died suddenly and another 6 had acute or Original Research Article British Journal of Medicine & Medical Research, 4(7): 1448-1459, 2014 1449 rapidly worsening symptoms. Three sudden deaths were due to dissection or rupture with haemopericardium and tamponade. Five cases had concomitant congenital heart defects. Conclusion: Sinus of Valsalva aneurysms are an uncommon cause of morbidity and mortality in non-Marfan patients. They are associated with certain congenital heart defects. There is often associated aortopathy. Sudden death can be the first manifestation and is most commonly due to aneurysm rupture or aortic dissection into the pericardial space. Echocardiography is the investigation of choice for diagnosis and follow-up. Prompt surgical or percutaneous intervention has an excellent long-term outcome.

A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation

OBJECTIVE First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL. DESIGN Cross-sectional observational study. SETTING Twenty-four cardiology centers from 15 countries across five continents. PATIENTS Four hundred thirty-five adult patients with congenital heart disease (177 Fontan and 258 ASD/VSD) ages 18-83 years. OUTCOME MEASURES QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions Questionnaire, respectively. RESULTS Patients with a Fontan circulation reported lower QOL (Wald Z = -3.59, p = <.001) and more negative perceptions of their CHD (Wald Z = -7.66, p < .001) compared with patients with ASD/VSD. After controlling for demographics, anxiety, depressive symptoms, and New York Heart Association functional class, path analyses revealed a significant mediation model, αβ = 0.15, p = .002, 95% CI = 0.06-0.25, such that CHD diagnosis was indirectly related to QOL through illness perceptions. CONCLUSIONS The Fontan samples more negative perceptions of CHD were likely a reflection of life with a more complex defect. Illness perceptions appear to account for unique differences in QOL between groups of varying CHD complexity. Psychosocial screening and interventions may be important treatment components for patients with CHD, particularly those with Fontan circulations.

Red Flags for Maltese Adults with Congenital Heart Disease: Poorer Dental Care and Less Sports Participation Compared to Other European Patients—An APPROACH-IS Substudy

Studies in recent years have explored lifestyle habits and health-risk behaviours in adult congenital heart disease (ACHD) patients when compared to controls. The aim of this study was to investigate differences in lifestyle habits between Maltese and other European ACHD patients. Data on alcohol consumption, cigarette smoking, substance misuse, dental care and physical activity collected in 2013–2015 during “Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study” (APPROACH-IS) were analysed. Responses from 119 Maltese participants were compared to those of 1616 participants from Belgium, France, Italy, Norway, Sweden, Switzerland and the Netherlands. Significantly fewer Maltese patients with simple (Maltese 84.1% vs. European 97.5%, p < 0.001) and moderately complex CHD (Maltese 83.6% vs. European 97.4%, p < 0.001) brushed their teeth daily. Only 67.2% of Maltese with moderately complex disease had dental reviews in the previous year compared to 80.3% of Europeans (p = 0.02). Maltese patients with simple (Maltese 31.8% vs. European 56.1%, p = 0.002) and moderately complex lesions (Maltese 30.0% vs. European 59.2%, p < 0.001) performed less regular sport activities. Comparison by country showed Maltese patients to have significantly poorer tooth brushing and sports participation than patients from any other participating country. Alcohol consumption, cigarette smoking and substance misuse were not significantly different. This study highlights lifestyle aspects that Maltese ACHD patients need to improve on, which might not be evident upon comparing patients to non-CHD controls. These findings should also caution researchers against considering behaviours among patients in one country as necessarily representative of patients on the larger scale.

A first population‐based long‐term outcome study in adults with repaired tetralogy of Fallot in Malta

OBJECTIVES To determine overall and reintervention-free survival for repaired Maltese tetralogy of Fallot patients and to investigate the potential impact of gender, age at repair, genetic syndromes, previous palliation, and type of repair on these outcomes. DESIGN All 130 tetralogy of Fallot patients born before the end of 1997 included in the local database were extracted. Surgical repair type, age at repair and operative survival were analyzed among the 103/130 repaired patients. Kaplan-Meier survival analyses were performed on the 75 repair survivors with complete follow-up data (mean follow-up 26.37 ± 9.27 (range 9.95-51.21) years). RESULTS Patients born after 1985 were operated at a younger age (median 1.28 years) compared with patients born before 1985 (median 9.64 years) (P < .001). Transannular patch repair was the commonest operation among patients born after 1985 (43.90%), while repair without transannular patch use prevailed among those born before 1985 (66.13%). 90.24% of patients born after 1985 survived reparative surgery compared with 70.37% of those born before 1985. Of the 75 repair survivors, 7 (9.33%) died of cardiac causes and 22 (29.33%) needed reintervention during follow-up. Overall estimated mean survival was 45.56 years (95% CI 41.67-49.24) with estimated survival rate of 77.5% at 40 years from repair. Estimated mean reintervention-free survival was 37.71 years (95% CI 33.75-41.66) with estimated reintervention-free survival rate of 59.2% at 40 years. Patients with genetic syndromes had significantly lower overall survival after repair. Transannular patch repair was associated with significantly lower reintervention-free survival (median 32.37 years (95% CI 12.75-51.99)) compared with repair without transannular patch [median 44.21 years (95% CI 43.06-45.35); P = .03]. CONCLUSIONS Although survival after tetralogy of Fallot repair in contemporary patients is very good, cardiac death can occur at any stage and structural reintervention is common. Regular follow-up with imaging and rhythm monitoring remains of utmost importance in all patients.