Recurrent Pneumonia and a Normal Heart: Late Complication after Repair of Hemianomalous Pulmonary Venous Drainage—A Cautionary Tale

Hemianomalous pulmonary venous drainage with intact atrial septum is a rare congenital anomaly and reports of its surgical repair and the long-term complications related to the correction are only infrequently encountered in the literature. We report the case of a patient with hemianomalous pulmonary venous drainage and intact atrial septum who underwent surgical repair using a pericardial baffle and creation of an “atrial septal defect” aged 15 years. Dyspnoea and recurrent chest infections started 7 months after surgery when he was seen by a respiratory physician without cardiac followup. He presented again aged 28 years with a recurrent pneumonia investigated over 6 weeks and heart pronounced normal from examination and echocardiography. Correct diagnosis was made in Grown Up Congenital Heart (GUCH) clinic stimulating review of data and catheterisation with pulmonary artery angiography which confirmed it. We feel that this case highlights the importance of specialist care and followup for GUCH patients

Can the inevitable be prevented? : an analysis of loss to follow-up among grown-ups with congenital heart disease in Malta

Aims: To investigate the prevalence of loss to follow-up, factors predisposing to loss to follow-up and the outcome of recall into specialist care among grown-ups with congenital heart disease (GUCH) of moderate or severe complexity prior to the introduction of formal transition in Malta. Methods: Medical documentation for all live patients with tetralogy of Fallot, aortic coarctation/interrupted aortic arch, partial and complete atrioventricular septal defect, Fontan-type circulation and transposition of the great arteries in our institutional database aged ≥16 years was analysed to determine follow-up status. Patients lost to follow-up were recalled through a postal appointment. Ordinal logistic regression was used to analyse the effect of gender, CHD complexity, consistency of paediatric cardiology follow-up during childhood, number of cardiac surgical/interventional procedures and use of long-term cardiac medications on loss to follow-up. Results: Forty-one of 187 patients (21.9%) (27 males; 34 with moderate disease) had been lost to follow-up. Limited paediatric cardiology input (OR, 5.08; 95% CI, 1.77-14.63) (p=0.003), £1 surgical/interventional procedures (OR, 3.34; 95% CI, 1.09-10.26) (p=0.035) and no long-term cardiac medications (OR 7.34; 95% CI, 1.74-31.02) (p=0.007) were associated with higher risk of loss to follow-up. A positive response to recall was obtained from 33/41 (80.5%) patients. Significant cardiac morbidity was found in 5/33 (15.2%) patients upon reassessment. Conclusions: Loss to specialist follow-up occurs even in health systems with little perceived barriers to medical care. Consistent specialist input during all stages and patient and family education through formal transition can help ensure a smoother transfer to GUCH care.peer-reviewe

Joints, bones and congenital heart disease... a forgotten association?

A 20 year old Caucasian male, with a history of uncorrected cyanotic congenital heart disease, presented with a one year history of bone pains in the upper thighs, legs and forearms. The diagnosis of hypertrophic osteoarthropathy (HOA) was picked up on bone scintigraphy. HOA is usually associated with lung disease and the link with congenital heart disease has become a less frequently encountered association.peer-reviewe

Relationship between thyroid status and survival rates in patients presenting with acute coronary syndrome

Thyroid dysfunction is a relatively common and treatable disease. The aims of the study included investigating the frequency of thyroid dysfunction in patients presenting with acute coronary syndrome (ACS) in our unit, following them up for 8 years and assessing the impact of thyroid dysfunction on their long-term outcome. Thyroid dysfunction is common among patients presenting with ACS in our unit. Forty percent (n=36) of those included had abnormal thyroid function tests at presentation with ACS, with the most common condition being subclinical hypothyroidism. The mean age of all patients was 62.3 years and there was no difference between males and females. There was no correlation between age and TSH and age and T4 levels. Forty two percent (n=37) died by the end of the observation period. A Kaplan-Meier curve was performed to check for any differences in survival across thyroid dysfunction categories. We report shorter survival times for patients who are euthyroid at presentation with an ACS.peer-reviewe

Extrinsic compression of an anomalous left main coronary artery in a patient with pulmonary arterial hypertension presenting with myocardial injury

Extrinsic compression of the left main coronary artery (LMCA) caused by severe pulmonary arterial dilatation in the setting of pulmonary arterial hypertension (PAH) is a recognised entity. This can present with angina, cardiogenic shock, malignant arrhythmias or sudden cardiac death. We report the case of a 17-year-old female with a history of primary PAH who presented with acute chest discomfort, elevated biomarkers and ECG changes. Invasive and non-invasive imaging confirmed her diagnosis and identified an anomalous origin of the left main coronary artery and significant LMCA extrinsic compression by an enlarged main pulmonary artery (MPA). The abnormal anatomical location of the LMCA resulting from its anomalous origin could have further contributedto the risk of compression.peer-reviewe

The impact of maternal congenital heart disease on pregnancy outcomes in Malta - a retrospective study

Background: Most female patients with congenital heart disease (CHD) are becoming pregnant. Maternal CHD can have a negative impact on mother and foetus. This is the first study investigating pregnancy outcomes in Maltese grown-up congenital heart disease (GUCH) patients and one of few to compare these with outcomes in women without heart disease. Methods: Known GUCH pregnancies for the period of 2007-2014 were extracted from our database (GUCH cohort) and cardiovascular outcomes retrieved from hospital notes. A control cohort of 540 pregnancies in women without cardiovascular disease was generated through twenty-fold random matching based on subject age from among all pregnancies in Maltese nationals for the same 8-year period. Obstetric and offspring outcomes were compared between the two cohorts. Results: The GUCH cohort consisted of 27 pregnancies in 24 women. Only 1/27 patients (3.7%) had cardiovascular complications. Elective Caesarean sections were commoner (29.6% vs. 15.4%) and unassisted vaginal deliveries less frequent (51.9% vs. 64.6%) in the GUCH cohort (p=0.02). Obstetric complication rates were similar. GUCH women had smaller babies (median 3030g vs. 3230g; p=0.045) and showed a trend towards more small-for-gestational age babies (18.5% vs. 8.4%; p=0.08) and congenital malformations (7.4% vs. 2.4%; p=0.06). Conclusions: Despite the potential adverse effects of maternal CHD on mother and foetus, most pregnancies are uncomplicated and outcomes comparable to those in women without heart disease, particularly if baseline clinical status is good. Based on our findings, it is being proposed that prospective mothers be counselled about the possibility of having smaller infants.peer-reviewe

Aortic coarctation : never too late to diagnose, never too late to treat

We present two cases of severe aortic coarctation detected in adulthood and who underwent successful relief by transcatheter stent deployment using a new covered stent, Optimus® stent (AndraTec GmbH Koblenz, Germany). One patient is a 46-year old female with resistant arterial hypertension, in whom coarctation was suspected on a follow-up transthoracic echocardiogram for bicuspid aortic valve disease and subsequently confirmed on magnetic resonance imaging. The second patient is a 68-year old male whose coarctation was diagnosed incidentally at coronary angiography being performed as part of the preoperative work-up for severe aortic stenosis. Suprasternal views to assess aortic arch and flows in the proximal descending aorta should be a standard part of every transthoracic echocardiogram. Treatment of aortic coarctation should be considered irrespective of patient’s age.peer-reviewe

When three is not a magic number : a case of native triple-valve endocarditis caused by Streptococcus agalactiae

We present a case of a 47-year-old female who presented with septic shock and a hyperosmolar hyperketotic state, accompanied by episodes of unresponsiveness. As part of the extensive investigations which took place, a trans-oesophageal echocardiogram (TOE) revealed the presence of infective endocarditis in three native valves – the tricuspid valve, pulmonary valve and the aortic valve. Blood cultures showed evidence of bacteraemia with Streptococcus agalactiae. The hyperosmolar hyperketotic state was controlled after optimisation of insulin treatment and the patient’s condition was relatively stable for weeks with intravenous antibiotics. The patient’s condition started deteriorating after multiple episodes of septic embolisation from the pulmonary valve vegetation, leading to urgent mechanical aortic, tissue pulmonary and tricuspid valve replacements in a tertiary centre in the United Kingdom; as well as a dual-chamber pacemaker implantation for post-operative complete heart block (CHB). After arrival back to Malta, the postoperative sternotomy wound was becoming recurrently infected, eventually leading to wound breakdown and overwhelming sepsis. Despite optimal medical treatment, wound care and intensive care support, the patient suffered from multi-organ failure and then passed away.peer-reviewe

Patient-reported outcomes in the aging population of adults with congenital heart disease: results from APPROACH-IS.

The congenital heart disease (CHD) population now comprises an increasing number of older persons in their 6th decade of life and beyond. We cross-sectionally evaluated patient-reported outcomes (PROs) in persons with CHD aged 60 years or older, and contrasted these with PROs of younger patients aged 40-59 years and 18-39 years. Adjusted for demographic and medical characteristics, patients ≥60 years had a lower Physical Component Summary, higher Mental Component Summary, and lower anxiety (Hospital Anxiety and Depression Scale-Anxiety) scores than patients in the two younger categories. For satisfaction with life, older persons had a higher score than patients aged 40-59 years. Registration: ClinicalTrials.gov NCT02150603

Phenotypes of adults with congenital heart disease around the globe: a cluster analysis

Objective To derive cluster analysis-based groupings for adults with congenital heart disease (ACHD) when it comes to perceived health, psychological functioning, health behaviours and quality of life (QoL). Methods This study was part of a larger worldwide multicentre study called APPROACH-IS; a cross sectional study which recruited 4028 patients (2013–2015) from 15 participating countries. A hierarchical cluster analysis was performed using Ward's method in order to group patients with similar psychological characteristics, which were defined by taking into consideration the scores of the following tests: Sense Of Coherence, Health Behavior Scale (physical exercise score), Hospital Anxiety Depression Scale, Illness Perception Questionnaire, Satisfaction with Life Scale and the Visual Analogue Scale scores of the EQ-5D perceived health scale and a linear analogue scale (0–100) measuring QoL. Results 3768 patients with complete data were divided into 3 clusters. The first and second clusters represented 89.6% of patients in the analysis who reported a good health perception, QoL, psychological functioning and the greatest amount of exercise. Patients in the third cluster reported substantially lower scores in all PROs. This cluster was characterised by a significantly higher proportion of females, a higher average age the lowest education level, more complex forms of congenital heart disease and more medical comorbidities. Conclusions This study suggests that certain demographic and clinical characteristics may be linked to less favourable health perception, quality of life, psychological functioning, and health behaviours in ACHD. This information may be used to improve psychosocial screening and the timely provision of psychosocial care