Hypoparathyroidism and mortality after total thyroidectomy: A nationwide matched cohort study.

OBJECTIVE: Total thyroidectomy (TT) carries a risk of hypoparathyroidism (hypoPT). Recently, hypoPT has been associated with higher overall mortality rates. We aimed to evaluate the frequency of hypoPT and mortality in patients undergoing TT in Denmark covering 20 years. DESIGN: Retrospective Cohort study. PATIENTS AND MEASUREMENTS: Using population-based registries, we identified all Danish individuals who had undergone TT between January 1998 and December 2017. We included a comparison cohort by randomly selecting 10 citizens for each patient, matched on sex and birth year. HypoPT was defined as treatment with active vitamin D after 12 months postoperatively. We used cumulative incidence to calculate risks and Cox regression to compare the rate of mortality between patients and the comparison cohort. We evaluated patients in different comorbidity groups using the Charlson Comorbidity Index and by different indications for surgery. RESULTS: 7912 patients underwent TT in the period. The prevalence of hypoPT in the study period was 16.6%, 12 months postoperatively. After adjusting for potential confounders the risk of death due to any causes (hazard ratio; 95% confidence intervals) following TT was significantly increased (1.34; 1.15-1.56) for patients who developed hypoPT. However, subgroup analysis revealed mortality was only increased in malignancy cases (2.48; 1.99-3.10) whereas mortality was not increased when surgery was due to benign indications such as goitre (0.88; 0.68-1.15) or thyrotoxicosis (0.86; 0.57-1.28). CONCLUSIONS: The use of active vitamin D for hypoPT was prevalent one year after TT. Patients with hypoPT did not have an increased risk of mortality following TT unless the indication was due to malignancy.

Risk of permanent social security benefits and overview of work participation among patients with obstructive sleep apnea.

BACKGROUND: In this nationwide study, we used Danish population registries to estimate the excess risk of receiving permanent social security benefits for patients with obstructive sleep apnea (OSA) and to track their labour force participation. METHODS: We identified all Danish citizens receiving a diagnosis of OSA between 1995 and 2015. As a reference cohort, we randomly selected 10 citizens for each patient, matched by sex and birth year. Using the Fine and Gray competing risk regression, we estimated the cumulative incidences of receiving permanent social security benefits. Cox proportional hazard models were used to compare the risk of receiving permanent social security benefits in patients with OSA compared to the reference cohort. The Danish Rational Economic Agents' Model (DREAM) database was used to identify the labour market status prior to diagnosis, at time of diagnosis, and after diagnosis. RESULTS: We identified 48,168 patients with OSA. A total of 12,413 (25.8%) patients with OSA had received permanent social security benefits, compared with 75,812 (15.7%) individuals in the reference cohort. Patients with OSA had a significantly increased risk of receiving permanent social security benefits when compared with the reference cohort (hazard ratio, 1.95; 95% CI, 1.88-2.02; and subhazard ratio, 1.92; 95% CI, 1.85-1.98). Work participation was lower for OSA patients compared to references at all time-points. CONCLUSION: Patients with OSA have a moderately increased risk of receiving permanent social security benefits in Denmark after controlling for available confounders.

Adults with small, unrepaired atrial septal defects have reduced cardiac index during exercise.

OBJECTIVES: Small, unrepaired atrial septal defects are considered a benign lesion with good prognosis. Recently, clinical and register-based studies discovered increased long-term mortality and morbidity. The nature of these findings is not fully understood. Therefore, MRI was performed to evaluate cardiac function at rest and during exercise. METHODS: Adults with open or spontaneously closed atrial septal defects and healthy, matched controls underwent MRI for evaluation of cardiac chamber volume. Quantitative flow scans measured blood flow in the ascending aorta and the proximal pulmonary artery at rest and during increasing supine exercise. RESULTS: In total, 15 open defects (39 ± 11 years) and 15 matched controls (38 ± 12 years) were included, along with 20 spontaneously closed (36 ± 13 years) and 20 controls (36 ± 11 years). Cardiac chamber volumes and flow measurements at rest were comparable between groups, as were heart rates and workloads during exercise. At maximal exercise, open defects reached 31% lower cardiac index and had 38% higher retrograde flow in the pulmonary artery than their controls, p < 0.01. Shunt ratio remained unchanged during exercise, 1.2 ± 0.2. Closed defects reached 18% lower cardiac index, p = 0.02, with comparable pulmonary retrograde flow. Maximal cardiac index was inversely correlated with increasing age for patients only. CONCLUSION: Adults with a small, open or spontaneously closed atrial septal defects exhibit markedly lower exercise capacity compared with healthy peers. Moreover, open defects exhibit higher retrograde flows with increasing exercise. Finally, increasing age is related to poorer results in patients but not healthy controls. Longitudinal studies are necessary in order to determine potential accelerated worsening of physical capacity along with age-related changes in patients.

Obstructive sleep apnea and road traffic accidents: a Danish nationwide cohort study.

STUDY OBJECTIVES: In this nationwide study, we investigate the risk and severity of all road traffic accidents in patients with obstructive sleep apnea (OSA). METHODS: We used the unique Danish registries to identify all Danish citizens receiving a diagnosis of OSA between 1995 and 2015. As a reference cohort, we randomly selected 10 sex- and age-matched citizens for each patient. We used Poisson regression to calculate the incidens rate ratio (IRR) for all road traffic accidents (motor vehicle, bicycle, and pedestrian) in both groups, and Cox proportional regression analysis to compare risk of first motor vehicle accident. Lastly, we used Fischers' Exact test to compare severity of motor vehicle accident between the two groups- RESULTS: We identified 48,168 patients with OSA, covering up to 24 years of follow-up. Patients with OSA had an increased risk of road traffic accidents when compared with the reference cohort (hazard ratio, 1.15; 95% CI, 1.10-1.20; IRR: 1.19; 95% CI, 1.14-1.29), especially motor vehicle accidents (hazard ratio, 1.29; 95% CI, 1.18-1.39; IRR 1.30; 95% CI, 1.20-1.42). The risk of accidents as pedestrian or bicyclist were not increased. Further, patients with OSA had a tendency to be involved in more severe motor vehicle accidents. CONCLUSIONS: This is the first nationwide study to estimate the risk of all road traffic accidents in patients with OSA. Our estimates show that patients with OSA have an increased risk of motor vehicle accidents, and greater severity of accidents, when compared with a large reference cohort.

Obstructive sleep apnea and risk of suicide and self-harm: a Danish Nationwide Cohort Study.

STUDY OBJECTIVES: In this nationwide study, we used the unique Danish registries to estimate the risk of suicide and deliberate self-harm in patients with obstructive sleep apnea (OSA). METHODS: We identified all Danish citizens receiving a diagnosis of OSA between 1995 and 2015. As a reference cohort, we randomly selected 10 citizens for each patient, matched by sex and birth year. Using the Fine and Gray competing risk regression, we estimated the cumulative incidences of suicide, and Cox proportional regression analysis was used to compare the risk of suicide and deliberate self-harm in patients with OSA with the reference cohort. RESULTS: We identified 48 168 patients with OSA. A total of 135 patients had died by suicide, compared with 999 suicides in the reference cohort. Patients with OSA had an increased risk of dying by suicide when compared with the reference cohort (hazard ratio, 1.29; 95% CI = 1.07% to 1.55%; and subhazard ratio, 1.23; 95% CI = 1.10% to 1.45%). We identified 1004 events of self-harm among patients with OSA, and 5270 events in the reference group. The overall risk of self-harm was increased in patients with OSA when compared with the reference group (hazard ratio, 1.28; 95% CI = 1.19% to 1.37%). CONCLUSIONS: This is the first study to estimate the risk of suicide and deliberate self-harm in patients with OSA. We found that patients with OSA have an increased risk of both suicide and deliberate self-harm when compared with a large reference cohort, thereby highlighting the importance of a mental health screening in these patients.

Necrotising Sweet's syndrome: a rare presentation of Sweet's syndrome with unilateral painful swelling of the neck.

This case describes the first documented clinical presentation of Sweet's syndrome with unilateral swelling of the neck, severe pain and fever. The clinical and radiological manifestation resembled necrotising fasciitis and the patient underwent acute neck dissection. The patient was ultimately diagnosed with a new subtype of Sweet's syndrome called necrotising Sweet's syndrome, and quickly recovered after treatment with intravenous administration of prednisolone.

Acute airway obstruction due to spontaneous intrathyroidal haemorrhage.

A woman in her mid-80's collapsed while home due to a sudden onset of swelling on her neck compromising the airway. She was intubated on-site and brought to the trauma-centre where she was diagnosed with an intrathyroidal bleeding with compression of the trachea. A subacute left sided thyroid lobectomy was performed, giving immediate relief to the displaced and compressed airway. The patient was discharged after 3 days. The final histopathological examination revealed a microfollicular adenoma with extensive bleeding.

Risk of Lifetime Psychiatric Morbidity in Adults With Atrial Septal Defect (from a Nation-Wide Cohort).

In this nation-wide cohort study we report the first long-term results of the association between having a atrial septal defects (ASD) on psychiatric disorders and use of psychotropic agents. Through population-based registries we included Danish individuals born before 1994 who received an ASD diagnosis between 1959and 2013. We used Cox proportional hazards regression and Fine and Grey competing risk regression to estimate the risk of receiving a psychiatric diagnosis and use of psychotropic medicine compared with a gender and age matched background population cohort. In 2,277 patients with a median follow-up from ASD diagnosis of 23.4 years (range 0.2 to 59.3 years) we found ASD patients to have a higher risk of psychiatric disorders (adjusted hazard ratio [HR]: 3.9; 95% confidence interval [CI] 3.4 to 4.5) compared with the comparison cohort and a cumulative incidence of using psychotropic agents 30 years after the ASD diagnosis of 47.4% (95% CI: 40.3 to 55.1) in the ASD patients and 25.5%, (95% CI: 23.5 to 27.8) in the comparison cohort. Diagnosis of the ASD before the age of 15 years (adjusted HR: 3.4; 95% confidence interval: 2.0 to 4.0) and surgical correction of the defect (HR: 1.5 (95% CI: 1.2 to 1.8), p <0.0001) had a higher risk than those with an ASD diagnosis after the age of 15 years and those with transcatheter closure of the defect. In conclusion, ASD patients had increased long-term risk of psychiatric disorder and use of psychotropic agents compared with a gender and age matched general population controls.

Pacemaker and conduction disturbances in patients with atrial septal defect.

OBJECTIVE: To determine the prevalence of pacemaker and conduction disturbances in patients with atrial septal defects. DESIGN: All patients with an atrial septal defect born before 1994 were identified in the Danish National Patient Registry, and 297 patients were analysed for atrioventricular block, bradycardia, right bundle branch block, left anterior fascicular block, left posterior fascicular block, pacemaker, and mortality. Our results were compared with pre-existing data from a healthy background population. Further, outcomes were compared between patients with open atrial septal defects and atrial septal defects closed by surgery or transcatheter. RESULTS: Most frequent findings were incomplete right bundle branch block (40.1%), left anterior fascicular block (3.7%), atrioventricular block (3.7%), and pacemaker (3.7%). Average age at pacemaker implantation was 32 years. Patients with defects closed surgically or by transcatheter had an increased prevalence of atrioventricular block (p < 0.01), incomplete right bundle branch block (p < 0.01), and left anterior fascicular block (p = 0.02) when compared to patients with unclosed atrial septal defects. At age above 25 years, there was a considerably higher prevalence of atrioventricular block (9.4% versus 0.1%) and complete right bundle branch block (1.9% versus 0.4%) when compared to the background cohorts. CONCLUSIONS: Patients with atrial septal defects have a considerably higher prevalence of conduction abnormalities when compared to the background population. Patients with surgically or transcatheter closed atrial septal defects demonstrated a higher demand for pacemaker and a higher prevalence of atrioventricular block, incomplete right bundle branch block, and left anterior fascicular block when compared to patients with unclosed atrial septal defects.

Congenital Heart Disease and Risk of Suicide and Self-Harm: A Danish Nationwide Cohort Study.

Background In this nationwide study, we used the unique Danish registries to estimate the risk of suicide and deliberate self-harm in patients with congenital heart disease (CHD). Methods and Results We identified all Danish citizens receiving a diagnosis of CHD between 1977 and 2007. As a reference cohort, we randomly selected 10 citizens for each patient, matched by sex and birth year. Using the Fine and Gray competing risk regression, we estimated the cumulative incidences of suicide and self-harm, and Cox proportional regression analysis was used to compare the risk of suicide and deliberate self-harm in patients with CHD with the reference cohort. We identified 14 433 patients with CHD. Mean follow-up was 21.3 years, with a maximum follow-up of 42 years. Since the time of diagnosis, 2659 patients had died, with a median age of death of 23 years. A total of 15 patients had died by suicide, compared with 232 suicides in the reference cohort. Patients with CHD had a low and similar risk of dying by suicide when compared with the reference cohort (cause-specific hazard ratio, 0.81; 95% CI, 0.48-1.37; and subhazard ratio, 0.68; 95% CI, 0.41-1.16). We identified 336 events of self-harm among patients with CHD, and 3484 events in the reference group. The overall risk of deliberate self-harm was not increased in patients with CHD when compared with the reference group (subhazard ratio, 0.95; 95% CI, 0.85-1.06). Conclusions This is the first study to estimate the risk of suicide and deliberate self-harm in patients with CHD. We found that patients with CHD do not have an increased risk of suicide or deliberate self-harm when compared with a large reference cohort.

Pregnancy outcome in women with atrial septal defect: associated with in vitro fertilisation and pre-eclampsia.

Objective: To investigate in vitro fertilisation (IVF) in women with atrial septal defect (ASD), and to examine the maternal characteristics and outcome of pregnancy, as well as the fetal outcome of infants born by women with ASD. Methods: We used population-based registries in this nationwide cohort study, including Danish individuals born before 1994 who received an ASD diagnosis between 1959 and 2014. Patients were compared with a random reference sample (ratio of 10 citizens per patient) matched by sex and age. The Danish Medical Birth Register (DMBR) contains data on all pregnancies and births in Denmark from 1977 to present. Risk of IVF treatment as well as maternal, pregnancy and fetal outcomes were compared. Results: A total of 2277 Danish patients had a validated ASD diagnosis. Of these, 310 women were identified in the DMBR. Women with ASD had an increased risk of receiving IVF treatment (HR 3.14, 95% CI 2.1 to 4.7, p<0.0001), and a higher proportion of patients received IVF treatment when compared with the reference cohort (10.6% vs 3.2%; p<0.001). Furthermore, patients had more multiple births. Looking at singleton pregnancies (n=519), pre-eclampsia occurred more frequently in patients with ASD during pregnancy (6.7% vs 2.3%; p<0.001). Infants from mothers with ASD were found to have perinatal outcomes comparable to those of infants from the reference group. Conclusion: Women with ASD had an increased risk of and received more IVF treatment than the reference group. The outcome of pregnancy in these patients were generally uneventful, however, we did confirm that pre-eclampsia occurred more frequently.

Hidden burden of arrhythmias in patients with small atrial septal defects: a nationwide study.

Background: In recent Danish nationwide register-based study, adults with small, unrepaired atrial septal defects (ASD) have increased risk of pneumonia, atrial fibrillation (AF) and stroke. Moreover, they revealed higher mortality than the background population. Objective: In this nationwide study, we evaluate the hidden burden of atrial and ventricular arrhythmias in adult patients with a small, unrepaired ASD without a previous diagnosis of AF. Methods: All Danish patients, aged 18-65, diagnosed between 1953 and 2011 with an unrepaired ASD and no documented AF were invited for 7 days Holter-recording, echocardiography and 6 min walk test. The first 48 hours Holter-recording was completely analysed, while only AF was screened for throughout all 7 days. Furthermore, the entire patient group were characterised using the unique Danish registries. Results: A total of 151 patients (mean age 32 years) were included. Approximately 80% of the patients had spontaneous closure of their defect. Despite this, occult arrhythmias were frequent. The most common arrhythmia was supraventricular tachycardia (n=24, 16%) with non-sustained atrial arrhythmias in 21 patients and AF in two patients. A considerable number of patients had non-sustained ventricular tachycardia (n=12, 8%). Patients with ASD and tachyarrhythmias had increased right ventricular to left ventricular diastolic area in echocardiography and higher age when compared with ASD patients without arrhythmias. Conclusion: Adult patients with small, unrepaired ASD have a hidden burden of both atrial and ventricular tachyarrhythmias. The mechanism likely relates to the residua of previous right-heart volume overload and incomplete reverse remodelling. Our results support guidelines recommending continued follow-up of patients with small, unrepaired ASD.

Biventricular contractility during exercise in adults with small, unrepaired atrial septal defects.

BACKGROUND: Adult patients with a small, unrepaired atrial septal defect (ASD) have higher mortality and increased risk of several comorbidities compared to general population. Further, reduced submaximal and peak exercise capacity was recently demonstrated in these patients. The mechanisms behind these findings remain unexplained and, therefore, biventricular contractility during exercise was assessed by evaluating the force-frequency relationship in the same group of patients. METHODS: Adults patients with a small, unrepaired ASD and healthy age- and gender-matched controls were examined using echocardiography during supine bicycle exercise. Continuous tissue velocity Doppler was used to evaluate isovolumetric acceleration (IVA) and systolic velocities during an incremental workload protocol. All data were analyzed post hoc in a blinded fashion. RESULTS: We included 30 patients previously diagnosed with a small, unrepaired ASD (mean age 35 years, 63% female) and 25 controls (mean age 34 years, 64% female). Patients had similar values of IVA and systolic velocities at rest when compared with the healthy controls. Further, no differences in IVA was found at peak heart rate for neither the left ventricle (90 ± 39 vs 129 ± 68 cm/s2 , P = 0.1547) nor the right ventricle (128 ± 56 vs 154 ± 56 cm/s2 , 0.5691). There were no correlations between peak velocities and the lower exercise capacity previously reported in these patients. CONCLUSION: Adult patients with a small, unrepaired ASD have normal biventricular contractility at rest and during exercise when compared with healthy peers. Consequently, the pathophysiological mechanisms behind the impaired exercise capacity previously demonstrated in these patients remains unknown and will be a target for future work.

Small atrial septal defects are associated with psychiatric diagnoses, emotional distress, and lower educational levels.

OBJECTIVE: For the first time, we wish to assess the psychiatric burden in adult patients living with small, unrepaired atrial septal defects (ASD) using register-based data, combined with self-reported measures on levels emotional distress and educational status. DESIGN: A descriptive study using both the unique Danish registries and validated psychiatric questionnaires and scales, including: The Symptom Checklist, Whiteley-7, and Brief Illness Perception Questionnaire. PATIENTS: Adult patients with small, unrepaired ASD, diagnosed between 1953 and 2011. OUTCOME MEASURES: Number of register-based psychiatric diagnoses. Additionally, symptoms of anxiety, depression, somatization, health anxiety, illness perception, and levels of educational attainment compared to age- and gender-matched individuals from the reference group. RESULTS: We identified 723 Danish patients with a small, unrepaired ASD. Approximately 17% of the patients eligible for inclusion had one or more psychiatric diagnoses. The most common diagnoses were neurotic, stress-related, and somatoform disorders. A total of 140 patients (mean age 33 years) was included for examination of their mental health using psychiatric questionnaires and scales. Patients with small ASD had higher scores of depression and anxiety when compared to the reference group (0.57 ± 0.67 vs 0.39 ± 0.52; P < .001). Furthermore, patients reported more symptoms of somatization (0.51 ± 0.48 vs 0.34 ± 0.39; P < .001). Finally, a higher percentage of patients with an unrepaired ASD had no education beyond high school when compared to the reference group (33.3% vs 14.3%; P < .001) and a smaller proportion of patients had completed a short-cycle higher education (6.5% vs 16.1%; P = .002). CONCLUSION: Patients with a small, unrepaired ASD in adult life have more symptoms of anxiety, depression, and somatization compared to the reference group. Furthermore, fewer patients had no education beyond high school. These results support the guidelines that adults with small ASD warrants regular lifelong follow-up in specialized clinics aware of not only adverse somatic outcome but also rates of depression and anxiety.

Lifelong burden of small unrepaired atrial septal defect: Results from the Danish National Patient Registry.

BACKGROUND: Adult patients with small, unrepaired atrial septal defects (ASD) have higher late mortality than the background population. In this nationwide study, we characterize the late natural history of adults with small, unrepaired ASD. METHODS: Using the Danish National Patient Registry, we included all Danish patients, diagnosed between 1953 and 2011 with an unrepaired ASD. Additionally, all patients, aged 18-65, were invited for clinical testing. Patients also completed a general health survey for comparison with the general population. RESULTS: We identified 723 patients with a small unrepaired ASD. Since the time of diagnosis, 182 patients had died, with an average lifespan of 63 years. The most common cause of death was heart failure. Furthermore, ASD patients had a higher burden of chronic disease than the general population (38.2% vs. 26.9%; p = 0.005), particularly lung disease (3.6% vs. 0.9%; p = 0.008). A total of 153 patients (mean age 32 y) underwent additional testing. On echocardiography an open defect was verified in 19.6% (n = 30) of the patients, of which half subsequently underwent intervention. Interestingly, 6-minute walking distance was markedly reduced (p < 0.0001 compared to normative values) no matter whether the defect was open or closed by echocardiography. Finally, 25.5% of the patients often felt stressed or nervous as compared with 16.3% of the general population (p = 0.004). CONCLUSIONS: Patients with small, unrepaired ASD in adult life have reduced lifespan, more chronic diseases, impaired submaximal exercise capacity, and higher levels of stress than the general population. The current guidelines for intervention and follow-up may need to be reconsidered.

Small unrepaired atrial septal defects display impaired exercise capacity compared with healthy peers.

OBJECTIVE: Adult patients with small, unrepaired atrial septal defects have an increased risk of pneumonia, atrial fibrillation, and stroke. Furthermore, they have higher late mortality than the background population. The functional capacity is unknown in these patients. Therefore, our objective was to determine exercise capacity in adult patients diagnosed with an unrepaired atrial septal defect compared to healthy controls. DESIGN: A cross-sectional study. PATIENTS: Adult patients with small, unrepaired atrial septal defects, aged 18-65, diagnosed between 1953 and 2011. INTERVENTIONS: Cardiopulmonary exercise test was performed using an incremental bicycle test and gas exchange was measured using breath-by-breath technique. OUTCOME MEASURES: Primary outcome was peak oxygen uptake, secondary outcome was maximal workload and ventilatory anaerobic threshold. RESULTS: We included 32 patients previously diagnosed with a small, unrepaired atrial septal defect and 16 healthy, age- and gender-matched controls (age 36.3 ± 13 years). Patients were divided into two groups based on whether the atrial septal defect was open (age 36.3 ± 11 years) or spontaneously closed (age 36.8 ± 14 years) since time of diagnosis. No differences in demographic characteristics or weekly exercise levels were found. Both patient groups reached lower peak oxygen uptake (open: 31.7 ± 11 mL/kg/min; spontaneously closed: 29.7 ± 6.9 mL/kg/min) compared with controls (42.6 ± 6.1 mL/kg/min; P = .0001). Workload (open: 2.6 ± 1.0 watt/kg; spontaneously closed: 2.5 ± 0.6 watt/kg) and aerobic capacity (open: 21.4 ± 8.7 mL/kg/min; spontaneously closed: 22.5 ± 6.5 mL/kg/min) was also poorer in patients compared to controls (workload: 3.5 ± 0.5 watt/kg; P = .0006, aerobic capacity: 31.3 ± 6.8 mL/kg/min; P = .0007). CONCLUSION: Adult patients with a diagnosis of small, unrepaired atrial septal defect have significantly impaired exercise capacity when compared to healthy controls. The impairment was present even if, by the time of assessment, the defect had closed spontaneously. The pathophysiological mechanisms behind the impaired exercise capacity demonstrated in these patients remain unexplained and will be a target for future work.

Prognostic power of cardiopulmonary exercise testing in Fontan patients: a systematic review.

Objective: Exercise impairment is common in Fontan patients. Our aim is to systematically review previous literature to determine the prognostic value of exercise capacity in older adolescent and adult Fontan patients with respect to late outcome. Additionally, we reviewed the determinants of exercise capacity in Fontan patients and changes in exercise capacity over time. Methods: PubMed, CINAHL, Embase, The Cochrane Library and Scopus were searched systematically for studies reporting exercise capacity and late outcome such as mortality, cardiac transplantation and hospitalisation. Studies were eligible for inclusion if more than 30 patients were included and mean age was ≥16 years. Results: Four thousand and seven hundred and twenty-two studies were identified by the systematic search. Seven studies fulfilled the inclusion and exclusion criteria. The total number of patients was 1664 adult Fontan patients. There were 149 deaths and 35 heart transplantations. All eligible studies were retrospective cohort studies. The correlation between exercise capacity and late outcome was identified, and HRs were reported. Conclusion: In Fontan patients, the best predictors of death and transplantation were a decline in peak VO2, heart rate variables and exercise oscillatory ventilation. Peak VO2 was not strongly predictive of mortality or hospitalisation in Fontan patients. Several variables were strong and independent predictors of hospitalisation and morbidity.

Histopathological abnormalities in the central arteries and veins of Fontan subjects.

OBJECTIVE: Fontan circulations have obligatory venous hypertension, depressed cardiac output and abnormal arterial elastance. Ventriculovascular coupling is known to be abnormal, but the underlying mechanisms are poorly defined. We aim to describe the histopathological features of vascular remodelling encountered in the central arteries and veins in the Fontan circulation as a possible underlying pathological representation of abnormal ventriculovascular coupling. METHODS: Postmortemvasculature (inferior vena cava (IVC), superior vena cava (SVC), pulmonary artery (PA), pulmonary vein (PV) and aorta) of 13 patients with a Fontan circulation (mean age 29.9 years, range 9.0-59.8 years) and 2 biventricular controls (ages 17.9 and 30.2 years) was examined. RESULTS: IVC and SVC: Eccentric and variable intimal fibromuscular proliferation occurred in 11 Fontan subjects. There was variable loss of medial smooth muscle bundles with reciprocal replacement with dense collagenous tissue.PA: Similar intimal fibromuscular proliferation was seen; however, these intimal changes were accompanied by medial thinning rather than expansion, medial myxoid degeneration and elastic alteration.PV: The PVs demonstrated intimal fibroproliferation and disorganisation of the muscular media.Aorta: The aortic lamina intima was thickened, with associated fibromuscular proliferation and elasticisation. There was also moderate lymphocytic inflammation in the aortic wall. CONCLUSIONS: Vascular architectural remodelling is common in Fontan patients. The central veins demonstrate profound changes of eccentric intimal expansion and smooth muscle replacement with collagen. The pulmonary demonstrated abnormal intimal proliferation, and aortic remodelling was characterised by intima lamina thickening and a moderate degree of aortic wall inflammation.

Cardiac function in adults following minimally invasive repair of pectus excavatum.

OBJECTIVES: To study if minimally invasive repair of pectus excavatum (PE) in adult patients would improve cardiopulmonary function at rest and during exercise as we have found previously in young and adolescent patients with PE. METHODS: Nineteen adult patients (>21 year of age) were studied at rest and during bicycle exercise before surgery and 1 year postoperatively. Lung spirometry was performed at rest. Cardiac output, heart rate and aerobic exercise capacity were measured using a photo-acoustic gas-rebreathing technique during rest and exercise. Data are shown as mean ± standard deviation. RESULTS: Fifteen patients completed the 1-year follow-up. No significant differences were found in neither cardiac output (14.0 ± 0.9 l min at baseline vs 14.8 ± 1.1 l min after surgery; P = 0.2029), nor maximum oxygen uptake (30.4 ± 1.9 and 33.3 ± 1.6 ml/kg/min; P = 0.0940 postoperatively). The lung spirometry was also unchanged, with no difference in forced expiratory capacity during the first second. CONCLUSIONS: Correction of PE in adult patients does not improve the cardiopulmonary function 1 year after surgery as seen in children and adolescents.

Right ventricular outflow tract obstruction caused by a displaced pectus bar 30 months following the Nuss procedure.

Complications following the Nuss procedure for the correction of pectus excavatum later than 30 days are rare. We report of a 40-year old man who developed dyspnoea and was diagnosed with a pulmonary stenosis more than 2 years after undergoing the Nuss procedure. This was discovered to be caused by a displaced pectus bar. Following removal of the pectus bars, the symptoms along with the right ventricular outflow tract obstruction disappeared.