Prognostic impact of self-reported health on clinical outcomes in patients with heart failure.

AIMS: An in-depth understanding of the prognostic value of patient-reported outcomes (PRO) is essential to facilitate person-centred care in heart failure (HF). This study aimed to clarify the prognostic role of subjective mental and physical health status in patients with HF. METHODS AND RESULTS: Patients with HF were identified from the DenHeart Survey (n = 1499) and PRO data were obtained at hospital discharge, including the EuroQol five-dimensional questionnaire (EQ-5D), the HeartQoL, and the Hospital Anxiety and Depression Scale (HADS). Clinical baseline data were obtained from medical records and linked to nationwide registries with patient-level data on sociodemographics and healthcare contacts. Outcomes were all-cause and cardiovascular (CV) mortality, CV events, and HF hospitalization with 1- and 3-year follow-up. Analysing the PRO data on a continuous scale, a worse score in the following were associated with risk of all-cause and CV mortality after 1 year: the HeartQoL (adjusted hazard ratios (HRs) 1.91, 95% confidence interval (CI) 1.42-2.57 and 2.17, 95% CI 1.50-3.15, respectively), the EQ-5D (adjusted HRs 1.26, 95% CI 1.15-1.38 and 1.27, 95% CI 1.13-1.42, respectively), the HADS depression subscale (adjusted HRs 1.12, 95% CI 1.07-1.17 and 1.11, 95% CI 1.05-1.17, respectively), and the HADS anxiety subscale (adjusted HRs 1.08, 95% CI 1.03-1.13 and 1.09, 95% CI 1.04-1.15, respectively). Three-year results were overall in concordance with the 1-year results. A similar pattern was also observed for non-fatal outcomes. CONCLUSION: Health-related quality of life and symptoms of anxiety and depression at discharge were associated with all-cause and CV mortality at 1- and 3-year follow-up.

Patient-reported outcomes and medication adherence in patients with heart failure.

AIMS: Patient-reported outcome measures (PROMs) may predict poor clinical outcome in patients with heart failure (HF). It remains unclear whether PROMs are associated with subsequent adherence to HF medication. We aimed to determine whether health-related quality of life, anxiety, and depression were associated with long-term medication adherence in these patients. METHODS AND RESULTS: A national cohort study of Danish patients with HF with 3-year follow-up (n = 1464). PROMs included the EuroQol five-dimensional, five-level questionnaire (EQ-5D-5L), the HeartQoL and the Hospital Anxiety and Depression Scale (HADS). Patient-reported outcomes (PRO) data were linked to demographic and clinical data at baseline, and data on all redeemed prescriptions for angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers/angiotensin receptor neprilysin inhibitors (ACEI/ARB/ARNI), ß-blockers, and mineralocorticoid receptor antagonists during follow-up. Medication non-adherence was defined as <80% of proportion of days covered. In adjusted regression analyses, lower health-related quality of life (EQ-5D and HeartQoL) and symptoms of depression (HADS-D) at discharge were associated with non-adherence. After 3 years of follow-up, lower health-related quality of life (EQ-5D) was associated with non-adherence for ACEI/ARB/ARNI [adjusted OR 2.78, 95% confidence interval (CI): 1.19-6.49], ß-blockers (adjusted OR 2.35, 95% CI: 1.04-5.29), whereas HADS-D was associated with non-adherence for ACEI/ARB/ARNI (adjusted OR 1.07, 95% CI: 1.03-1.11) and ß-blockers (adjusted OR 1.06, 95% CI: 1.02-1.10). CONCLUSION: Lower health-related quality of life and symptoms of depression were associated with non-adherence across HF medications at 1 and 3 years of follow-up. Person-centred care using PROMs may carry a potential for identifying patients at increased risk of future medication non-adherence.

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.

Two cases of "stone heart" with fatal outcome.

The "stone heart" syndrome is a rare but often fatal complication of cardiac surgery associated with hypertrophy of the myocardium. The mechanisms behind the syndrome are not fully understood. In this case report, we describe two cases of stone heart in newborn girls. Both girls were born with congenital heart abnormalities including ventricular septum defects (VSD), hypertrophy of the myocardium and patent arterial duct (PDA), which was prenatally diagnosed. In each of the two cases, the stone heart became evident immediately after initiating cardiopulmonary bypass, and ended fatally.

Closure of a patent foramen ovale in patients with platypnoea-orthodeoxia: a rare and overlooked cause of dyspnoea and hypoxaemia.

OBJECTIVE: Platypnoea-orthodeoxia is a rare cause of dyspnoea and hypoxaemia induced by a change from supine to upright position resulting in veno-arterial shunting, usually through a patent foramen ovale. We aimed to describe our experience with closure of a patent foramen ovale in patients with platypnoea-orthodeoxia, and to increase awareness of this diagnosis. DESIGN: Eight patients (mean age 63 ± 12 years) were identified from hospital records between 2000 and 2013. RESULTS: The underlying diseases were aortic aneurism (n = 3), cardiac transplantation (n = 2), left ventricular dilatation (n = 1), pulmonary fibrosis (n = 1), and recurrent pleural/pericardial effusion (n = 1). At presentation, mean oxygen saturation was 81 ± 8% in the upright position. All patients underwent transthoracic and transoesophageal echocardiography. Pulmonary pressures were normal at rest in all patients. A right-to-left shunt through a patent foramen was demonstrated after changing posture from supine to sitting. Closure of the patient foramen ovale was performed uncomplicated in all patients. Mean oxygen saturation post-procedure was 96 ± 2%. All but one patient experienced marked symptom relief. CONCLUSION: The majority of patients with platypnoea-orthodeoxia can benefit from treatment of the underlying condition. In the present cohort, patients underwent uncomplicated catheter-based closure of the patient foramen ovale with subsequent improvement of oxygen saturation and symptom improvement.

Adverse events in transcatheter interventions for congenital heart disease: a population-based long-term study.

OBJECTIVE: Few studies have reported procedure complications or adverse events for transcatheter interventions in unselected congenital heart disease cohorts. We report our 23-year experience with transcatheter interventions in congenital heart disease and examine predictors for adverse events. DESIGN AND SETTING: This study was a population-based cohort study of children and adults with congenital heart disease, covering a population of 3 million inhabitants in western Denmark. Adverse events were subdivided into 5 levels according to their severity. Procedure-type risk category, age, weight, and year of procedure were included in multivariate logistic models to identify risk factors for adverse events. RESULTS: Between 1990 and 2012, 1595 patients had 1878 catheter-based interventions performed. We identified 241 adverse events, corresponding to 13% of the procedures; 58 (3%) were considered to be of high severity. During the study period, there was an increase in number of procedures per year (P < .001) and a decrease in frequency of adverse events (P = .01). Procedure-type categories 3 and 4 had increased risk of adverse events when compared with category 2, with odds ratios of 1.7 (95% confidence interval [CI]: 1.2-2.3) for category 3 and 2.3 for category 4 (95% CI: 1.4-3.6). Age and weight at catheterization were not independently associated with adverse events. CONCLUSIONS: We found an increase in number of procedures over time and a decrease in frequency of adverse events. Higher procedure-type risk categories were associated with increased risk of adverse events.

Menstrual bleeding after cardiac surgery.

OBJECTIVES: We investigated whether open-heart surgery with the use of extracorporeal circulation has an impact on menstrual bleeding. METHODS: The menstrual bleeding pattern was registered in fertile women undergoing open-heart surgery in 2010-12. Haematocrit and 24-h postoperative bleeding were compared with those of men undergoing cardiac surgery. RESULTS: Women (n = 22), with mean age of 36 (range 17-60) years, were operated on and hospitalized for 4-5 postoperative days. The mean preoperative haematocrit was 40% (range 32-60%), and mean haematocrit at discharge was 32% (range 26-37%). Mean postoperative bleeding in the first 24 h was 312 (range 50-1442) ml. They underwent surgery for atrial septal defect (n = 5), composite graft/David procedure (n = 4), pulmonary or aortic valve replacement (n = 6), myxoma (n = 2), mitral valvuloplasty (n = 2), ascending aortic aneurysm (n = 1), aortic coarctation (n = 1) and total cavopulmonary connection (n = 1). Unplanned menstrual bleeding (lasting 2-5 days) was detected in 13 (60%) patients. Of them, 4 were 1-7 days early, 4 were 8-14 days early, 3 were 1-7 days late and 2 had menstruation despite having had menstrual bleeding within the last 2 weeks. None had unusually large or long-lasting menstrual bleeding. Ten women took oral contraceptives, 8 of whom had unexpected menstrual bleeding during admission. Men (n = 22), with a mean age of 35 (range 17-54) years, had mean bleeding of 331 (range 160-796) ml postoperatively, which was not statistically significantly different from the women's. The mean preoperative haematocrit was 40% (range 29-49%) among men, while haematocrit at discharge was 32% (28-41), not significantly different from that seen in the female subgroup. CONCLUSIONS: Menstrual bleeding patterns are disturbed by open-heart surgery in the majority of fertile women. Nevertheless, the unexpected menstrual bleeding is neither particularly long-lasting nor of large quantity, and the postoperative surgical bleeding is unaffected. We recommend that information about menstrual cycle disturbances related to cardiac surgery be provided, but no special precautions be taken, when operating on women of fertile age.

Progressive cyanosis following Kawashima operation: slow resolution after redirection of hepatic veins.

Progressive cyanosis often develops following Kawashima operation in patients with left atrial isomerism, interrupted inferior vena cava and hepatic veins draining to the atria. Knowledge on the timing and extend of resolution following hepatic venous redirection is sparse. A girl developed progressing cyanosis following Kawashima operation at the age of ten months. Arterial oxygen saturations at rest dropped to 60-65%. Surgical redirection of hepatic veins into the cavopulmonary circulation at the age of three years had no immediate effect. However, arterial oxygen saturations increased gradually over nine months to 90-93% and 95-100% after three years.

Hypertrophic cardiomyopathy in childhood: disease natural history, impact of obstruction, and its influence on survival.

BACKGROUND: We investigated the natural history, outcomes of myectomy, and impact on survival of obstructive hypertrophic cardiomyopathy (HCM) in childhood. METHODS: All 120 children diagnosed with HCM between 1971 and 2006 were studied. Available echocardiograms (n=685) were incorporated in regression analyses adjusted for repeated measures. Multiphase parametric models and competing risks methodology were used to define outcomes. RESULTS: Left ventricular outflow tract (LVOT) obstruction (peak gradient>30 mm Hg) developed in 61 (50%) of the cohort. Onset of obstructive features occurred during 2 discrete periods of risk; either during infancy (within 3 years of age), or otherwise later during early adolescence and beyond. Among children with obstructive HCM, the following 2 distinct groups were apparent: (1) those with peak LVOT gradients less than 65 mm Hg showed hemodynamic improvement (p<0.001) with medical strategies; and (2) those with peak gradients greater than 65 mm Hg instead had accelerated septal hypertrophy (p<0.001) with progression of peak gradients (p<0.01), and therefore underwent myectomy. Myectomy restored the gradient (mean 57 mm Hg reduction, 95% confidence interval 25 to 88, p<0.01) to nonobstructive levels. Furthermore, peak gradients and septal hypertrophy did not progress thereafter; they instead mirrored the natural history of nonobstructive HCM. Overall, in our experience, obstructive disease (or need for myectomy) did not influence late risk of death compared with children with nonobstructive HCM. CONCLUSIONS: Obstructive HCM is phenotypically heterogeneous in childhood. Peak gradients less than 65 mm Hg respond well to nonsurgical management. The patient subset with higher gradients (>65 mm Hg) instead responds poorly and early myectomy should be pursued. Reassuringly, late survival in this cohort was not compromised by development of obstructive symptoms.

Survival and morbidity following congenital heart surgery in a population-based cohort of children--up to 12 years of follow-up.

OBJECTIVES: The Risk Adjusted Classification for Congenital Heart Surgery can predict early mortality. However, the relation to long-term outcome in terms of mortality and morbidity is unknown. DESIGN: We did a population-based follow-up study of 801 children undergoing congenital heart surgery between 1996 and 2002. All patients were followed from surgery until death or January 1, 2008. Operations were classified according to the Risk Adjusted Classification for Congenital Heart Surgery. Each patient was matched by age and sex with 10 population controls. Cox regression analysis, area under the receiver operator curve and competing risk analysis were used for the analyses. RESULTS: Overall follow-up was 99.6%. The distribution of the Risk Adjusted Classification for Congenital Heart Surgery was: Category one 20%, category two 37%, category three 27%, category four 8%, category five 0% and category six 2%. Overall survival after a median follow-up of 8.2 years was 86% (95% confidence interval: 83-88%), with 54 early deaths occurring within 30 days after surgery and 57 late deaths. Long-term survival in those who were alive 30 days after surgery was 92% (90-94%); ranging from 98% (93-100%) in risk category one to 33% (5-68%) in category six. Survival overall and beyond 30 days was lower in each risk category than in controls (P < .001). During follow-up, 124 (15%) patients had new operations and 106 (13%) catheter-based interventions. These events were more frequent in category three, four, and six compared with category one, with no difference between category one and two. The area under the receiver operator curve for long-term mortality was 0.81 (95% confidence interval 0.75-0.87). CONCLUSIONS: Children operated for congenital heart disease have impaired survival and often undergo new operations or catheter-based interventions. The risk of these events is related to the surgical complexity according to the Risk Adjusted Classification for Congenital Heart Surgery.

[Pregnancy after Mustard operation for transposition of the great arteries]. / Graviditet efter Mustards operation for transpositio vasorum.

INTRODUCTION: Previously, the Mustard operation was the preferred surgical technique in patients with transposition of the great arteries. After this procedure the anatomical right ventricle remains the systemic ventricle, which entails long-term complications, especially heart failure. The Mustard-operated patients are now adults, and pregnancy has become an important issue. We assessed pregnancy and delivery data in a complete cohort of female patients who had previously undergone a Mustard procedure at Aarhus Hospital in the 1971-1991 period. The women giving birth were compared with those who did not. MATERIAL AND METHODS: Information on diagnosis, procedure, functional status, pregnancy, delivery and congenital heart disease in the off-spring were found in registers (The National Register of Health, The National Birth Register) and in medical records. Questionnaires were mailed to the 16 surviving women over 15 years of age. RESULTS: Four women gave birth to nine children. Two women temporally had heart symptoms during pregnancy and delivery, but this caused no objective changes. The women who had children were older than those who did not. None of the children had congenital heart disease nor were they small for their gestational age. CONCLUSION: Pregnancy and delivery are well-tolerated in the vast majority of Mustard-operated women with no deterioration in functional class during or after pregnancy and delivery.

Medium-term follow-up of mechanical valves inserted in children.

OBJECTIVE: We reviewed our experience with mechanical valves inserted between 1988 and 2002 in children aged 15 years or younger. METHODS: Hospital files were extracted retrospectively. Follow-up was completed by March 2005. RESULTS: Of 41 patients, we inserted a valve in atrioventricular position in 27 children, having a median age of 3.1 years, ranging from 0.4 to 14.5 years, and in aortic position in the remaining 14, having a median age of 13.5 years, and a range from 7.0 to 14.9 years. For the valves inserted in atrioventricular position, the underlying disease was congenital in 23, rheumatic in two, post-endocarditic in one, and Marfan's syndrome in one. Mean follow-up was 7.7 years, with standard deviation of 5.3, giving a total follow-up of 209 patient years. Mortality at 30 days was 7%, and survival was 73% at up to 16 years follow-up. Events related to anticoagulation were seen in 3 patients, corresponding to 1.4% per patient year. In 6 patients (22%), heart block ensued which required implantation of a pacemaker treatment, and 5 patients (19%) had reoperations. For the implantations in aortic position, the underlying disease was congenital in 13, stenosis in 10 and insufficiency in three, and post-endocarditis in one. Mean follow-up was 6.8 years, with standard deviation of 4.6, giving a total of 95 patient years. We lost one patient within 30 days (7.7% mortality), and survival was 77% at up to 13 years follow-up. There were no incidents of thrombosis, nor events related to anticoagulation, but one patient (7%) needed insertion of a pacemaker due to a perioperative heart block, and one (7%) required new valvar replacement. CONCLUSIONS: Although preferably avoided, mechanical valves can be implanted in children with an acceptable mortality and morbidity, and good long-term results.

[Risk adjustment for surgery of congenital heart disease--secondary publication]. / Risikoscore ved operation for medfødt hjertesygdom--sekundaerpublikation.

Risk adjustment for specialties covering many diagnoses is difficult. The Risk Adjusted classification for Congenital Heart Surgery (RACHS-1) was created to compare the in-hospital mortality rate of groups of children undergoing surgery for congenital heart disease. We applied the classification to the operations performed at Skejby Sygehus (1996-2002) and found that RACHS-1 can be used to predict the in-hospital mortality rate and length of stay in the intensive care unit in a Danish center for congenital heart surgery. The mortality rate was similar to that reported by larger centers.

The RACHS-1 risk categories reflect mortality and length of stay in a Danish population of children operated for congenital heart disease.

OBJECTIVE: The Risk Adjusted classification for Congenital Heart Surgery (RACHS-1) was created in order to compare in-hospital mortality for groups of children undergoing surgery for congenital heart disease. The method was evaluated with two large multi-institutional data sets-the Paediatric Cardiac Care Consortium (PCCC) and Hospital Discharge (HD) data from three states in the USA. The RACHS-1 classification was later applied to a large German paediatric cardiac surgery population in Bad Oeynhausen (BO), where it was found that the RACHS-1 categories were also associated with length of stay. We applied the RACHS-1 classification to the 957 operations performed during January 1996 to December 2002 at Skejby Sygehus, Denmark and we examined the association between the RACHS-1 categories, in-hospital mortality and length of stay in the Intensive Care Unit. METHODS: The operations were classified according to the six RACHS-1 categories by matching the procedure of each patient with a risk category. The ability of the RACHS-1 classification to predict mortality in our population was examined by estimating the area under the receiver operator characteristic (ROC) curve. Likelihood ratio chi(2) tests were used to compare the distribution of RACHS-1 categories and the distribution of mortality with PCCC, HD and BO. Linear regression was used to examine the correlation between the RACHS-1 categories and length of stay in the Intensive Care Unit. RESULTS: The RACHS-1 category frequencies in our population were: category 1: 18.4%, category 2: 37.4%, category 3: 34.6%, category 4: 8.2%, category 5: 0% and category 6: 1.5%. The overall ability of the RACHS-1 classification to predict in-hospital mortality (area under the ROC curve 0.741; 95% confidence interval=0.690; 0.791) was equal to the findings from larger populations. We found no differences in the category specific mortality when comparing with the larger reported series. There was a positive association between RACHS-1 category and length of stay in the Intensive Care Unit. CONCLUSIONS: The RACHS-1 classification can also be used to predict in-hospital mortality and length of stay in the Intensive Care Unit in a small volume centre.