Late Cardiac Complications Research Articles

Temporal trend in hospitalization among patients with congenital heart disease in Denmark: a nationwide study

Abstract Background The survival rate of patients with congenital heart disease (CHD) had increased over the last decades due to improved management. However, the increased survival rate may potentially impact the number of occurrences of late cardiac complications as well as non-cardiovascular diseases requiring hospitalization, and hence warrants further study on this area. Purpose To delineate the temporal trend in hospitalization rate and length of stay (LOS) among patients with major and minor CHD in Denmark from 1977 to 2018 and to investigate the causes of hospitalization. Methods A Danish nationwide, population-based registry study including patients with CHD diagnosis from 1977 to 2018. Patients were categorized as either major or minor CHD, and by age groups (<1, 1-4, 5-17, 18-39, 40-64, ≥65 years). Hospitalization rate was reported as incidence per 100 person-years. Results In this study, 23,386 patients were included with 121,653 hospital admissions. From 1977-1981 to 2012-2018, the overall all-cause hospitalization rate decreased for both major (51.7 [CI: 48.0 -55.7] to 32.8 [CI: 31.0 - 34.7]) and minor CHD patients (28.4 [CI: 26.0 - 31.0] to 22.6 [CI: 21.1 - 24.1]). Median LOS for major CHD decreased from 5 days (IQR: 2 – 11) to 2 days (IQR: 1 – 6), and for minor CHD from 4 days (IQR: 2 – 8) to 2 days (IQR: 1 – 5). However, the trends when looking at specific age groups were different from the trend of overall all-cause hospitalization rate. Figure 1 shows that hospitalization rate had increased significantly for <1-year-olds for major CHD, whereas an increased trend was observed for <1, 40-64 and ≥65 years-olds for minor CHD. In addition, the trends for specific age groups when looking at the most common causes were also different from the trend of all-cause hospitalization rate. For both major and minor CHD, Table 1 shows that the hospitalization rate for <1-year-olds due to respiratory diseases had increased over time, while the hospitalization rate for 40-64-year-olds due to arrythmia had also increased over time. For ≥65-year-olds, significant increase in hospitalization rate for arrythmia was only observed for minor CHD. Conclusion Lower hospitalization rate for major and minor CHD after 1 year old suggests that early intervention of CHD has improved their morbidity during childhood and early adulthood. However, the increased hospitalization rates among <1 year-olds and the elderly suggest that more focus are needed in preventing non-cardiovascular diseases and late cardiac complications for the respective age groups.Figure 1Table 1

Does Pacemaker Implantation After Surgical Aortic Valve Replacement Impact Long-Term Morbidity and Mortality? A Focused Review.

Permanent pacing remains a serious complication that can occur in the postoperative period of surgical aortic valve replacement. The reported incidence is variable, and there are many perioperative factors that have been linked with a greater need for permanent pacing. Permanent pacing can also be associated with late lead-related and cardiac complications that can affect late outcome. However, the degree of late dependence on pacemakers is varied, and some studies have shown that a substantial proportion of patients do not need long-term pacing. Some groups have found that permanent pacing was associated with a negative impact on long-term survival in these patients. A common finding among these studies is that the groups of patients with pacemakers had higher preoperative surgical risk and comorbidity status. This makes it difficult to establish whether permanent pacing on its own represents a risk factor for late mortality or whether it is simply a marker that reflects the higher complexity and comorbidities in this group of patients.

Heart failure in childhood cancer survivors\u2014a systematic review protocol

BackgroundOver the past decades, the survival rate for childhood cancer has greatly improved. However, the risk of late cardiac complications after cancer treatment remains high. Previous studies have shown that the risk for heart failure among childhood cancer survivors is significantly higher than that observed in varying control populations. The aim of this systematic review is to identify, critically appraise, and synthesize existing population-based studies reporting on the frequency of heart failure, both the incidence and prevalence, that may develop after treatment for childhood cancer.MethodThe following databases will be searched from their inception date until May 17, 2021: MEDLINE, Embase, Scopus, CINAHL, CAB International, AMED, Global Health, PsycINFO, Web of Science, and Google Scholar. Population-based studies reporting on the incidence and/or prevalence of heart failure after the treatment of any type of childhood cancer will be included. The screening of articles, data extraction, and quality assessment will be performed independently by two reviewers. The quality and risk of bias in the included studies will be assessed by using the Effective Public Health Practice Project tool. A narrative synthesis of the extracted data will be carried out, and for studies that are sufficiently homogenous, a meta-analysis using random-effects models will be performed.DiscussionThis systematic review will provide a clearer picture of the epidemiology of heart failure after the treatment of childhood cancer. The collected data will be of value for future childhood cancer treatment protocols and will offer guidance for posttreatment cardiac surveillance among survivors.Systematic review registrationPROSPERO CRD42021247622. Registered on April 28, 2021. This protocol follows the structure of the recommendation of the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols (PRISMA-P).

Adjuvant combined therapy with trastuzumab in patients with HER2‑ positive breast cancer and cardiac alterations: implications for optimal cardio‑oncology care.

Recently, the prognosis of patients with HER2‑positive breast cancer (BC) has improved significantly owing to the use of combined treatment modalities. However, systemic treatment is as-sociated with increased risk of cardiotoxicity. We aimed to assess subclinical cardiac alterations during the final stage of adjuvant com-bined therapy, that is, trastuzumab therapy (TT), as potential predictors of late cardiac complications in patients with HER2‑positive BC. We enrolled 251 patients with HER2‑positive BC treated with a radical local therapy, adjuvant chemotherapy (anthracyclines or anthracyclines + taxanes), and immunotherapy (trastuzumab). Patients underwent 6 echocardiographic examinations: at baseline, during TT, and after TT, with assessment of left ventricular ejection fraction (LVEF), degree of valvular regurgitation, and cardiac chamber diameters. Valvular fibrosis (28.4% of patients) was associated with older age, hypertension at baseline, and a higher degree of regurgitation during TT. Reduced LVEF, greater regurgitation, and larger cardiac chamber diameters were noted during TT. The patients who received higher anthracycline doses showed a greater degree of aortic insufficiency and a larger right ventricular diameter. Reduced LVEF during TT was associated with radiotherapy or chemotherapy and the degree of valvular regurgitation. Significantly larger diameters were observed in older patients and in those with comorbidities at baseline, high body mass index, and regurgitation. Asymptomatic subclinical cardiac alterations during TT may predict late cardiac complica-tions; however, longer follow‑up is necessary to confirm this hypothesis. Patients with HER2‑positive BC should be closely monitored for possible cardiac alterations during and after therapy to ensure optimal care and guide therapeutic decision‑making.

DISSEMINATED TUBERCULOUS PERICARDITIS IN A YOUNG ADULT WITH SICKLE CELL DISEASE

The incidence of tubercle bacilli (TB) pericarditis has decreased in the modern era. Clinical evidence of TB may be subtle, making the diagnosis tricky especially in vulnerable patients with sickle cell disease (SCD). Late cardiac complications include constrictive pericarditis and increased

Gender-Related Differences in Sickle Cell Disease in a Pediatric Cohort: A Single-Center Retrospective Study.

Sickle cell disease (SCD) is one of the most common monogenic disease worldwide. The incidence of SCD is not strictly gender-related as it is transmitted as an autosomal recessive disorder. In particular, the gender-related differences in pediatric SCD are not well-characterized. To address this matter, we retrospectively analyzed the clinical records of 39 pediatric patients with a diagnosis of SCD (hemoglobin SS genotype) focusing on gender differences analyzing various aspects of the disease and comprising both acute symptoms and late complications. We found various gender-related differences in our pediatric population. In particular, pain crisis frequency per year was significantly increased in the male population with a mean number of crisis per year of 1.6 vs. 0.6 in the female population (p = 0.04). Also, severe complications (both infectious and cardiovascular) were mostly found in the male population. SCD-related late cardiac complications were observed mainly in the male population (p = 0.04). Our data support the hypothesis that gender could play a role in determining the clinical course of SCD, even though further studies are needed to assess the exact weight of its influence over the course of the disease. The higher morbidity in males is a well-known feature of SCD in adults and these findings have been only partially studied in the pediatric population. These differences have, in adults, been attributed to hormonal variations found in the two sexes after puberty. In a pediatric population, other factors must be responsible for these discrepancies. These findings suggest that gender could be a valuable factor in the risk stratification of these patients at diagnosis, and possibly guide therapeutic decisions, with the final aim of personalizing the therapy.

Changes of the Heart Valves in the Long Term After Chemoradiotherapy According to Different Protocols for Hodgkin's Lymphoma in Children and Adolescents.

Purpose: The purpose of our work was to study late cardiac complications after treatment for Hodgkin's lymphoma (HL) in children and adolescents. Methods: Sixty-seven patients were examined in the long term (>5 years) after chemoradiotherapy for HL according to two different programs of treatment (groups I and II). Mean total doses of radiotherapy (RT) to the mediastinum were 37.2 and 28.9 Gy, respectively. The status of the heart was assessed at the mean age of 22.7 years with electrocardiography (ECG) and echocardiography (EchoCG). Mean terms of follow-up were 16.4 and 9.5 years for group I and group II, respectively. Results: Incidence of ECG changes was equal between the groups (88% and 90%). The prevalence of signs of valvular calcifications and fibrosis was 70.9% after mediastinal doses ≥30 Gy, and 16.6% after lower doses (p = 0.002). Those changes led to considerable valvular dysfunction in four patients. EchoCG signs of pulmonary hypertension were seen in 33.3% patients of group I versus 4.8% in group II (p = 0.047). Pericardial effusion was observed in 7.4% and 5.1%, respectively (p = 1.0). Left ventricular ejection fraction decreased slightly only in two patients (one in each group). Conclusions: The RT mediastinal dose level is the important risk factor of late heart complications. Nevertheless, the differences in the rate and severity of those complications between the groups should be viewed with caution because of differences in the age at baseline and in follow-up terms. The survivors of HL should undergo life-long regular examinations of the heart status.

Cardiovascular complications of oncological therapies

Cardiovascular diseases and oncological pathology are, unfortunately, the main causes of mortality and disability in the world. Fortunately, prevention through serious programs, but also the early detection and new therapies already have exceptional results in many forms of neoplasms. It is not less true that pharmaceutical research and improvement of the equipment intended for diagnosis and radiotherapy have led to the categorical modification of the chances of the oncological pa­tients. The prognosis in the medium and long term is in­com­pa­ra­bly better for many of these patients, the sur­vi­val being frequent and lasting. With this reality of survival of the majority of patients, there arose the need to recognize the late cardiac complications of oncological therapy. Good oncological survival on the one hand, but also the risk of late complications of the specific therapy oblige us to suspect and recognize the remote complications of the oncological treatment. The family doctor, who coordinates the care of his patients, is the one who must know these risks, to suspect and to monitor the possible complications. Oncological patients who have undergone radiation or chemotherapy (with potential cardiotoxicity), or both, should be actively monitored oncologically and cardiologically for many years. The cardiovascular complications of oncology therapy may be silent and serious, but they may benefit from effective treatment if detected early.

Lessons from prenatal diagnosis and in utero transfer of fetuses with transposition of the great arteries

Prenatal diagnosis of transposition of the great arteries (TGA) reduces neonatal mortality and morbidity. The mortality of prenatally diagnosed TGA remains scarcely described. To review the experience of prenatal diagnosis and in utero transfer of fetuses with TGA to assess the limits of this program and to describe outcomes of prenatally diagnosed TGA. From 1999 to 2017, we reviewed all cases of prenatally diagnosed TGA born at our institution. Files were analyzed for discordances between prenatal and postnatal diagnosis, status at birth, neonatal management, and outcomes. In total, 748 fetuses with TGA (470 simple, 278 complex) were delivered at our institution. Discordance between pre- and postnatal diagnosis was noted in 14.8% with two third having consequence on surgical treatment. Median term was 39 WG. The proportion of premature delivery (< 37WG) was 5.3%, the majority being late-preterm (> 34 WG). Fourteen percent were intubated in the delivery room and 1.7% required resuscitation maneuvers. Sixty-four percent had a Rashkind procedure that was done in 15% of cases in the delivery room. Fifty-five percent received PGE1 infusion and 38% had a Rashkind and PGE1 infusion. There were 9 deaths before surgery (1.2%) with two not related to the TGA. A fourth experienced neonatal complications including mainly respiratory distress requiring ventilation, infection, necrotizing enterocolitis, pulmonary hypertension and iatrogenic events. Surgical mortality was 1.8% (4 simple TGA and 10 complex). Seven additional deaths occurred during the first year follow-up, 6 being related to late cardiac complications. Overall survival at one year was 96%. Prenatal diagnosis and in utero transfer of fetuses with TGA does not eliminate the risk of preoperative mortality. The proportion of immediate distress is high and the need for intensive care in the delivery room is frequent. Finally, preoperative mortality accounts for a third of the one-year mortality in this population.

Combined mechanical mitral valve replacement and transmitral myectomy for hypertrophic obstructive cardiomyopathy treatment: An experience of over 20 years

BackgroundAlthough transaortic septal myectomy (TASM) is recognized as a standard procedure for treating hypertrophic obstructive cardiomyopathy (HOCM), occasionally the left ventricle (LV) intracavitary gradient remains postoperatively because of this technically demanding procedure. Mitral valve replacement (MVR) is sometimes chosen as an alternative option, but data on its long-term outcomes are lacking. Methods and resultsBetween 1991 and 2016, 29 patients [age, 14–82 (mean 58.9±15.9) years; 22 female patients (75.9%)] underwent combined mechanical MVR and transmitral myectomy. Of these, six patients had undergone MVR following a second cardiac arrest because of the residual LV outflow gradient or residual mitral regurgitation following TASM. Concomitant TASM was performed in 13 patients. The LV intracavitary gradient at rest assessed by transthoracic echocardiography significantly decreased postoperatively (16.8±19.1mmHg vs. 107.4±52.5mmHg, p<0.0001). Actuarial freedom rates from cardiac death were 92.8%, 89.0%, and 80.1% at 5, 10, and 15 years postoperatively, respectively. Sudden death occurred in three of the four patients who died of late cardiac complications. None of these patients with sudden death had implantable cardioverter-defibrillators. Most patients had maintained their LV end-diastolic dimension at <50mm for 10–15 years postoperatively. Actuarial freedom rates from hospitalization for heart failure were 87.7%, 82.2%, and 54.8% at 5, 10, and 15 years postoperatively, respectively. Occurrence rates of cerebral hemorrhage and infarction were 0.6% per patient-year and 1.3% per patient-year, respectively. ConclusionsCombined mechanical MVR and myectomy is an effective procedure to eliminate the LV intracavitary gradient in patients with HOCM. Although this procedure remains a viable option in certain situations, optimal medical treatment and close clinical follow-up along with the cooperation between cardiac surgeons and cardiologists are necessary to achieve favorable long-term outcomes.

Evaluation of the Motion Range using Surface Mapping System during the Deep Inspiration Breath Hold for Left-Sided Breast Cancer Patients

Radiation Therapy (RT) for left-sided breast cancer can cause late cardiac complications. Deep Inspiration Breath Hold (DIBH) during RT has been accepted as an effective approach to reduce the irradiated heart volume as well as reduce the motion of the tumor bed. However, the range of motion /reproducibility of each breath hold (BH) has not been quantitatively studied. Here, we report the results of our evaluation of the motion recorded using a surface guided radiotherapy (SGRT) solution during DIBH manipulated by Active Breath Control (ABC) device. Patients with stages I/II left-sided breast cancer received 3D conformal treatments on a radiation therapy system. For each of the tangential beams, radiation was delivered during 1 or 2 breath-holds with the duration controlled by ABC device. In addition, each patient’s motion was monitored by a CatalystHD (C-RAD, Uppsala, Sweden) SGRT solution which consists of 3 high resolution camera/detector pairs. The motion within each breath hold duration at deep inspiration level, which was represented by a virtual tracking point on patient’s left chest wall, was recorded using the CatalystHD. The range of motion during Inter-BH and Intra-BH were statistically analyzed. A total of 47 ABC treatment sessions including 136 BHs were evaluated for five patients. All patients received tangential radiation to the breast/chest wall (typical doses 180 cGy x 28 or 266 cGy x 16); and all patients received a tumor bed boost. The range of motion varied from patient to patient. The patient with the least chest-wall motion during BH showed a 2.5(±0.7)-mm Inter-BH motion and 1.3(±0.4)-mm Intra-BH motion. On the other hand, the most Inter-BH motion and Intra-BH motion observed for one patient was 13.0(±5.4) mm and 9.4(±3.2) mm. Among 136 BHs, the average of Inter-BH motion and Intra-BH motion were 7.1(±4.9) mm and 4.7(±3.9) mm, respectively. The average duration of DIBH was 13.5(±7.8) s. The range of the motion during DIBH manipulated by ABC is not negligible. It varies between patients and even varies among the different BHs of the same patient on the same day. It should be monitored carefully to ensure appropriate target dose coverage and to prevent the development of late cardiac complications. Additionally, the surface guided system, with proper coaching and the assist of a video-based coaching system (e.g. goggles), can be used as an alternative to monitor the respiratory motion at DIBH in left breast cancer patients who have difficulty breathing through a mouthpiece or who cannot tolerate the ABC device.

Late Cardiac Complications of Sulfur Mustard Poisoning in 38 Iranian Veterans.

It was aimed to investigate possible late cardiac effects of Sulfur mustard (SM) exposure in Iranian veterans. Thirty-eight veterans with late complications of SM exposure were investigated. Clinical history, physical examinations, 12 leads electrocardiography and transthoracic echocardiography were performed. Computed tomography coronary angiography (CTCA) was performed as clinically indicated for angiographic assessment and patients were stratified according to the CTCA findings. Incomplete right bundle branch block and right axis deviation were detected in 3 (7.9%) and 4 (10.5%) cases, respectively. Mean value of left ventricular ejection fraction was 55.7 ± 2.9%. Different degrees of right ventricular dilation was observed in seven (18.4%) patients. All the patients showed mild to moderate degrees of tricuspid regurgitation. Increased pulmonary artery pressure (PAP) was detected in 16 (42.1%) patients. Out of 18 patients who underwent CTCA, non-obstructive and obstructive coronary artery disease (CAD) were observed in three (16.66%) and eight (44.44%) patients, respectively. CAD was stratified to single vessel (5.5%), two vessels (27.8%) and three vessels disease (11.1%). Mean coronary artery calcium score was 50.91 ± 115.58. SM has cardiovascular toxicity, as a delayed complication of this chemical warfare poisoning.

Tamibarotene As Maintenance Therapy for Acute Promyelocytic Leukemia Improved Long Term Relapse-Free Survival: 7-Year Results from a Randomized Controlled Trial, JALSG-APL204

Background: Tamibarotene, a new synthetic retinoid, displays (i) approximately 10-fold increased potency over ATRA at inducing in vitro differentiation of NB-4 cells (ii) enhanced chemical stability compared with ATRA (iii) low affinity for cellular RA-binding protein. The clinical efficacy of tamibarotene for the treatment of acute promyelocytic leukemia (APL) has also been reported. A prospective randomized controlled study to evaluate tamibarotene by comparison to ATRA was carried out for maintenance therapy of APL in JALSG APL204 (Shinagawa et al, 2014). The 4-year-relapse free survival (RFS) did not differ between patients treated with ATRA or tamibarotene. However, an improved efficacy of tamibarotene in high-risk patients was suggested, which warrants further investigation. Here, we evaluate the long-term outcome of the study. Patients and Methods: Patients enrolled in this study were newly diagnosed with APL and documented cytogenetic and/or molecular evidence of t(15;17)/ PML-RARA . The age of the patients ranged between 15 and 70 years and the ECOG performance status was between 0 and 3. For remission induction therapy, ATRA was administered to all patients at a daily dose of 45 mg/m2 until complete remission (CR). The chemotherapy protocol varied depending on the initial leukocyte count and blast count in the peripheral blood. In brief, patient groups were defined as: leukocytes Results: A total of 347 patients were enrolled in the study. Of the 344 eligible patients, 319 (93%) achieved CR. After completing three courses of consolidation therapy, 269 patients underwent maintenance random assignment; 135 patients were assigned to ATRA, and 134 patients were assigned to tamibarotene. The mean follow-up of patients alive and relapse-free at the date of last contact was 7 years. The 7-year RFS rate was 84% for the ATRA arm and 93% for the tamibarotene arm (p=0.031) (Fig.1). When the analysis was restricted to 52 high-risk patients with an initial leukocyte count ≥ 10,000/µl, the difference was more prominent (62% vs 89%, p=0.034) (Fig.2). The 7-year RFS of induction treatment for Group A (92 cases) was 91%, Group B (38 cases) 92%, Group C (52 cases) 75% and Group D (87 cases) 91% (p=0.005). Both treatments were generally well tolerated. Secondary hematopoietic disorders were observed in 12 cases, malignancies in 9 cases, late cardiac complications (grade 3 or more) in 5 cases. However, there was no significant difference in terms of these complications between the two treatment groups. Conclusions: Maintenance therapy with tamibarotene was effective at decreasing the relapse rate in APL patients by comparison to ATRA at the 7-year observation point. In particular, tamibarotene was significantly more effective than ATRA for high risk patients with leukocytes ≧10,000/μl. These results could lead to a new strategy for the treatment of high risk patients, which is one of the recent priority issues in the treatment of APL. Disclosures Takeshita: Chugai Pharmaceutical Co., Ltd.: Research Funding; Astellas Pharma Inc.: Research Funding; Phizer Japan Inc.: Research Funding; Takeda Pharmaceutical Co Ltd: Research Funding. Asou: Chugai Pharmaceutical Co., Ltd.: Research Funding; Astellas Pharma Inc.: Research Funding. Ueda: KAINOS LABORATORIES INC: Membership on an entity9s Board of Directors or advisory committees; Abrynx nv: Membership on an entity9s Board of Directors or advisory committees; Elli LiLLY Japan KK: Other: Clinical Trial; Takeda PharmaceuticalCompany Limited: Other: Clinical TRial; Otsuka Pharmaceutical Co Ltd: Other: Clinical Trial; Celgene KK: Other: Clinical Trial; Symbio Pharmaceutical Limited: Other: Clinical Trial; AstellasPharma Inc: Other: Clinical Trial; Eisai Co. Ltd: Other: Clinical Trial. Fujita: Chugai Pharmaceutical: Honoraria. Usui: Nippon Shinyaku Pharmaceutical Co: Research Funding. Kobayashi: Pfizer, Ohtsuka, Astellas, Ariad: Research Funding. Kiyoi: Kyowa Hakko Kirin Co., Ltd.: Research Funding; Nippon Shinyaku Co., Ltd.: Research Funding; ONO Pharmaceutical Co., Ltd.: Research Funding; Takeda Pharmaceutical Co., Ltd.: Research Funding; Nippon Boehringer Ingelheim Co., Ltd.: Research Funding; Meiji Seika Pharma Co.,Ltd.: Research Funding; FUJIFILM Corporation: Patents & Royalties, Research Funding; Bristol-Myers Squibb: Honoraria, Research Funding; Eisai Co., Ltd.: Research Funding; Celgene Corporation: Consultancy, Research Funding; Chugai Pharmaceutical Co., Ltd.: Research Funding; Astellas Pharma Inc.: Consultancy, Research Funding; Zenyaku Kogyo Co., Ltd.: Research Funding; Phizer Japan Inc.: Honoraria, Research Funding; MSD K.K.: Research Funding; Novartis Pharma K.K.: Research Funding; Sumitomo Dainippon Pharma Co., Ltd.: Research Funding; JCR Pharmaceuticals Co.,Ltd.: Research Funding. Atsuta: Otsuka Pharmaceutical Co., Ltd.: Honoraria. Naoe: Otsuka Pharmaceutical Co.,Ltd.: Honoraria, Research Funding; Nippon Boehringer Ingelheim Co.: Honoraria, Research Funding; Fujifilm Corporation: Patents & Royalties, Research Funding; Dainippon Sumitomo Pharma Co.,LTD.: Research Funding; Astellas Pharma Inc.: Research Funding; Toyama Chemikal Co.,Ltd.: Research Funding. Miyazaki: Nippon Shinyaku: Honoraria.

Evaluating Cardiac Biomarkers after Chemotherapy and Proton Therapy for Mediastinal Hodgkin Lymphoma.

Late cardiac complications from thoracic radiation of patients with Hodgkin lymphoma are of great concern. The authors investigated whether cardiac biomarkers could identify patients with early cardiac damage from thoracic radiation. Following completion of anthracycline-based chemotherapy, 14 patients with stage I-IIIA mediastinal Hodgkin lymphoma were enrolled on an IRB-approved prospective trial and agreed to serum evaluation of troponin, CK-MB, and BNP before beginning radiation, after completing radiation, and every 3 to 6 months for 5 years, during follow-up or until relapse. Among the 14 patients, median follow-up for the entire cohort was 5 years. No evidence in abnormal values in troponin or CK-MB was observed among the patient cohort. BNP levels demonstrated statistically significant higher values at various follow-up time points. Higher levels of BNP were observed among patients receiving higher anthracycline doses (>250 mg), but not among patients with higher mean heart radiation doses. In the first 5 years after treatment, no rise in CK-MB or troponin was identified. BNP levels significantly increased following treatment, with larger increases among patients who received higher doses of anthracyclines. Further investigation is warranted.

Cardiac findings in children with juvenile Dermatomyositis at disease presentation

BackgroundJuvenile Dermatomyositis (JDM) is a pediatric vasculopathy characterized primarily by skin and muscle involvement. Cardiac findings have been reported in children with JDM but have rarely been investigated in detail.MethodsWe aimed to describe the relevant clinical and laboratory cardiac findings of a cohort of patients with JDM, followed at one centre, at disease diagnosis.ResultsWe performed a retrospective review of 105 patients with JDM, followed from 1991 to 2007. Six of 70 patients (9%, 6% of the entire cohort) had abnormal electrocardiographic (ECG) findings, while 26 of 54 patients (48%, 25% of the entire cohort) had abnormal echocardiographic (echo) findings. Many of these findings were either mild or unlikely to be a result of JDM.ConclusionsOur findings suggest that cardiac abnormalities at JDM disease onset are frequently seen, but are rarely significant findings due to disease; however, JDM patients should be considered for screening for cardiac disease as late cardiac complications are well recognized.

A cluster randomized trial of a transition intervention for adolescents with congenital heart disease: rationale and design of the CHAPTER 2 study.

BackgroundThe population of adolescents and young adults with congenital heart disease (CHD) is growing exponentially. These survivors are at risk of late cardiac complications and require lifelong cardiology care. However, there is a paucity of data on how to prepare adolescents to assume responsibility for their health and function within the adult health care system. Evidence-based transition strategies are required.MethodsThe Congenital Heart Adolescents Participating in Transition Evaluation Research (CHAPTER 2) Study is a two-site cluster randomized clinical trial designed to evaluate the efficacy of a nurse-led transition intervention for 16–17 year olds with moderate or complex CHD. The primary endpoint is excess time to adult CHD care, defined as the time interval between the final pediatric cardiology appointment and the first adult CHD appointment, minus the recommended time interval between these appointments. Secondary endpoints include the MyHeart score (CHD knowledge), Transition Readiness Assessment Questionnaire score, and need for catheter or surgical re-intervention. Participants are enrolled in clusters based on week of attendance in the pediatric cardiology clinic. The intervention consists of two one-hour individualized sessions between a cardiology nurse and study participant. Session One focuses on knowledge of the participant’s CHD, review of their cardiac anatomy and prior interventions, and potential late cardiac complications. Session Two focuses on self-management and communication skills through review and discussion of videos and role-play. The study will recruit 120 participants.DiscussionMany adolescents and young adults experience a gap in care predisposing them to late cardiac complications. The CHAPTER 2 Study will investigate the impact of a nurse-led transition intervention among adolescents with CHD. Fidelity of the intervention is a major focus and priority. This study will build on our experience by (i) enrolling at two tertiary care programs, (ii) including a self-management intervention component, and (iii) evaluating the impact of the intervention on time to ACHD care, a clinically relevant outcome. The results of this study will inform pediatric cardiology programs, patients and policy makers in judging whether a structured intervention program provides clinically meaningful outcomes for adolescents and young adults living with CHD.Trial registrationClinicalTrials.gov ID NCT01723332

Erythema marginatum rheumaticum

A 12-year-old boy presented with high grade intermittent fever for 15 days with polyarthralgia involving the large joints of his extremities for last 5 days. His parents also noticed appearance of a pinkish coloured non-pruritic rash on the back of the trunk about a week after the onset of fever which resolved spontaneously only to reappear on the extensor surface of his right leg in the morning of the day of presentation. On examination, the patient was febrile with an oral temperature of 102 °F. The large joints of his extremities were not swollen but were tender on palpation. The rash was non-tender and non-pruritic, having elevated and reddened edges with a flat and clear centre (Fig. 1). Rest of the systemic examination was normal except sinus tachycardia. Complete blood count was normal except for raised ESR (90 mm), C-reactive protein (53 mg/L) and Anti-streptolysin O titre (754 IU). EKG was done and it revealed sinus tachycardia only. Trans-thoracic echocardiography documented thickened mitral valve leaflets with mild mitral regurgitation. Throat culture grew Streptococcus pyogenes. The patient was diagnosed to have acute rheumatic fever (ARF) based on the revised Jones criteria, treated symptomatically and was advised prophylactic benzathine penicillin G for secondary prevention until adulthood. Fig. 1 Erythematous annular papule over right lower limb with central clearing and serpentines spreading edges. Erythema marginatum, a form of annular erythema, is very uncommon in children experiencing the first attack of ARF [1]. The typical lesions have central clearing and serpenginous spreading edges and often are unnoticed by the patient or parent because they are painless and non-pruritic. It becomes even more difficult to detect the rash in dark-skinned people, explaining its rare occurrence (less than 1%) in ARF patients in non-white populations [2], [3]. Fever triggers the development of this rash, which perhaps explains the evanescent nature of the rash. Physicians should be careful not to overlook this rare but useful clinical manifestation of ARF, particularly in patients with subclinical valvular involvement to avoid potential late cardiac complications.

Pregnancy following atrial-switch repair.

One of the early repairs for complete transposition of the great arteries was the atrial-switch (Mustard or Senning) operation. This operation involved the re-direction of venous return using inter-atrial baffles. Patients with atrial-switch operations are at risk of developing a number of late complications of pregnancy, including dysfunction of the subaortic right ventricle, atrioventricular valve (AV) regurgitation, and arrhythmias. Pregnancy imposes additional haemodynamic stress on the subaortic right ventricle that can result in ventricular remodelling and deterioration in the systolic function of the ventricle. There is limited data on late effects of pregnancy on the subaortic right ventricle and the AV valve. Understanding the impact of pregnancy on maternal cardiac health is important in order for women with heart conditions to make informed pregnancy decisions. An early study examining pregnancy outcomes in this population (Guedes et al. J Am Coll Cardiol 2004;44:433–37) suggested that deterioration in subaortic ventricular function and AV valve regurgitation was common after pregnancy; however, no control group was available to determine whether this deterioration represented the natural history of the disease or the accelerated effects of pregnancy on the suboaortic right ventricle. In 2013, Bowater et al. (Congenit Heart Dis 2013;8:302–7) reported on 18 women (31 pregnancies) over a 4–5 year period and compared outcomes with 23 female and 28 male controls. In the postpartum period, pregnant women showed significant deterioration in both subaortic ventricular function and New York Heart Association (NYHA) functional status. In late follow-up, although the deterioration of subaortic ventricular function in all three groups was similar, the difference in NYHA functional status persisted in pregnant women when compared with controls. In this issue of BJOG, Cataldo et al. compare 10–year outcome data for 23 women (34 pregnancies) with atrial-switch repair with 15 female controls. In late follow-up, all women remained free of clinical heart failure and the rates of late maternal cardiac complications and deterioration of cardiac function were similar for both groups (cases versus controls). When compared with controls, however, pregnant women had a greater incidence of worsening AV valve regurgitation. In addition to adverse maternal cardiac outcomes, fetal and neonatal complications were notable, as were the high rates of labour induction for cardiac deterioration or concerns for fetal wellbeing, highlighting the impact of maternal health on fetal outcomes. Although the current study is limited by its retrospective nature, small numbers, and qualitative assessment of ventricular function, it contributes to our understanding of the late outcomes after pregnancy in this population. Although pregnancy in women with atrial-switch operations is usually well tolerated, this study and others suggest that, over the long-term, the haemodynamic stress of pregnancy may have adverse late effects on the heart, including worsening NYHA functional class and AV valve regurgitation. None declared. Completed disclosure of interests form available to view online as supporting information. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Simple Method to Estimate Mean Heart Dose From Hodgkin Lymphoma Radiation Therapy According to Simulation X-Rays

To describe a new method to estimate the mean heart dose for Hodgkin lymphoma patients treated several decades ago, using delineation of the heart on radiation therapy simulation X-rays. Mean heart dose is an important predictor for late cardiovascular complications after Hodgkin lymphoma (HL) treatment. For patients treated before the era of computed tomography (CT)-based radiotherapy planning, retrospective estimation of radiation dose to the heart can be labor intensive. Patients for whom cardiac radiation doses had previously been estimated by reconstruction of individual treatments on representative CT data sets were selected at random from a case-control study of 5-year Hodgkin lymphoma survivors (n=289). For 42 patients, cardiac contours were outlined on each patient's simulation X-ray by 4 different raters, and the mean heart dose was estimated as the percentage of the cardiac contour within the radiation field multiplied by the prescribed mediastinal dose and divided by a correction factor obtained by comparison with individual CT-based dosimetry. According to the simulation X-ray method, the medians of the mean heart doses obtained from the cardiac contours outlined by the 4 raters were 30 Gy, 30 Gy, 31 Gy, and 31 Gy, respectively, following prescribed mediastinal doses of 25-42 Gy. The absolute-agreement intraclass correlation coefficient was 0.93 (95% confidence interval 0.85-0.97), indicating excellent agreement. Mean heart dose was 30.4 Gy with the simulation X-ray method, versus 30.2 Gy with the representative CT-based dosimetry, and the between-method absolute-agreement intraclass correlation coefficient was 0.87 (95% confidence interval 0.80-0.95), indicating good agreement between the two methods. Estimating mean heart dose from radiation therapy simulation X-rays is reproducible and fast, takes individual anatomy into account, and yields results comparable to the labor-intensive representative CT-based method. This simpler method may produce a meaningful measure of mean heart dose for use in studies of late cardiac complications.

Image-guided radiotherapy for cardiac sparing in patients with left-sided breast cancer.

Patients with left-sided breast cancer are at risk of cardiac toxicity because of cardiac irradiation during radiotherapy with the conventional 3-dimensional conformal radiotherapy technique. In addition, many patients may receive chemotherapy prior to radiation, which may damage the myocardium and may increase the potential for late cardiac complications. New radiotherapy techniques such as intensity-modulated radiotherapy (IMRT) may decrease the risk of cardiac toxicity because of the steep dose gradient limiting the volume of the heart irradiated to a high dose. Image-guided radiotherapy (IGRT) is a new technique of IMRT delivery with daily imaging, which may further reduce excessive cardiac irradiation. Preliminary results of IGRT for cardiac sparing in patients with left-sided breast cancer are promising and need to be investigated in future prospective clinical studies.