Search

Physicians who specialize in pulmonary arterial hypertension (PAH) care for patients facing a serious, life-limiting illness. Palliative care is underutilized in patients with PAH, and little is known about how best to provide palliative care to this patient population.Using a qualitative approach, assess physicians' perspectives on barriers and facilitators to the use of palliative care in PAH.Participants were board-certified pulmonologists and cardiologists recruited from the Pulmonary Hypertension Association's list of physician specialists and academic center websites. We performed one-on-one semi-structured interviews that were recorded, transcribed, and analyzed using thematic analysis.Twelve physicians participated in the study, with a median age of 48.5 years and 20.5 years of clinical experience caring for patients with PAH. We identified the following themes and associated barriers and facilitators to effective implementation of palliative care for patients with PAH: a tailored approach to the individual patient; a PAH-specialist-led culture of care; effective collaboration with palliative care clinicians; and limitations imposed by health systems.PAH physicians are open to palliative care for their patients and are willing to partner with palliative care clinicians to implement this effectively and in the right setting. Areas for targeted improvement in enhancing palliative care for patients with PAH exist, especially enhancing collaboration between PAH physicians and palliative care specialists and navigating barriers in health systems.

Background: While the incidence of pregnancy has increased among individuals with adult CHD, little has been described about considerations and experiences of patients with adult CHD regarding pregnancy. Objective: We aimed to explore patients’ motivations, concerns, and decision-making processes regarding pregnancy. Methods: In April 2019–January 2020, we conducted in-depth telephone interviews with patients (n = 25) with simple, moderate, or complex adult CHD, who received prenatal care at the University of Washington during 2010–2019 and experienced a live birth. Transcripts were analysed using thematic analysis. Results: Participants described motivations for pregnancy as both internal desires (motherhood, marriage fulfillment, biological connection, fetal personhood, self-efficacy) and external drivers (family or community), as well as concerns for the health and survival of themselves and the fetus. Factors that enabled their decision to maintain a pregnancy included having a desire that outweighed their perceived risk, using available data to guide their decision, planning for contingencies and knowing their beliefs about termination, plus having a trusted healthcare team, social support, and resources. Factors that led to insurmountable risk in subsequent pregnancies included desire having been fulfilled by the first pregnancy, compounding risk with age and additional pregnancies, new responsibility to an existing child, and reduced healthcare team and social support. Conclusions: Understanding individuals’ motivations and concerns, and how they weigh their decisions to become or remain pregnant, can help clinicians better support patients with adult CHD considering pregnancy. Clinician education on patient experiences is warranted.

Pregnancy risk assessment for patients with adult congenital heart disease (ACHD) must include physiologic and anatomic impacts. We aimed to determine whether maternal cardiac and pregnancy outcomes vary by disease severity defined according to the following 3 different classifications: ACHD anatomic severity, ACHD physiologic class, and modified World Health Organization (mWHO) class. Cardiac outcomes included a composite of arrhythmia, heart failure, stroke, and thromboembolism. Pregnancy outcomes included a composite of intrauterine growth restriction, preterm birth, preeclampsia, or postpartum hemorrhage. We employed generalized estimating equations to account for multiple pregnancies. Of the 245 pregnancies, 17.1% were preterm and 45.7% were cesarean deliveries. Cardiac hospitalizations occurred in 22.0% and arrhythmias in 12.7%. Cardiac outcomes tended to be more prevalent in people with more severe heart disease. Pregnancy outcomes were U-shaped or less prevalent in people with more severe disease. There was a 2.9-fold increased risk for the composite cardiac outcome for complex anatomy (adjusted incidence rate ratio 2.90, 95% confidence interval 1.08 to 7.81, p = 0.04), a 9.4-fold increased risk for physiologic class C or D (9.37, 1.28 to 68.79, p = 0.03), and a fourfold increased risk for mWHO class III or IV (3.99, 1.53 to 10.40, p = 0.005). There was a lower risk for the composite pregnancy outcome for mWHO class II or II to III (0.54, 0.36 to 0.79, p = 0.002) but no association with anatomy or physiology. In conclusion, physiologic class may be most accurately associated with adverse outcomes and therefore efforts to optimize hemodynamics before pregnancy may help to mitigate the risk.

Cor triatriatum sinistrum (CTS) is a rare congenital cardiac malformation in which the left atrium is divided by a fenestrated membrane, which can restrict blood flow and cause symptoms of congestive heart failure. Rarely, the condition can present in adulthood. This case report illustrates a case of sudden cardiac death (SCD) due to the sequelae of untreated CTS. To date, there are no reported cases of SCD attributable to CTS. LEARNING OBJECTIVES: Cor triatriatum sinistrum is among the rarest of congenital heart diseases. In this case report, we describe the prevalence, etiology, diagnosis, and management of this disease.

Little is known about policies and practices for patients undergoing Transcatheter Aortic Valve Replacement (TAVR) who have a documented preference for Do Not Resuscitate (DNR) status at time of referral. We investigated how practices across TAVR programs align with goals of care for patients presenting with DNR status.Between June and September 2019, we conducted semi-structured interviews with TAVR coordinators from 52/73 invited programs (71%) in Washington and California (TAVR volume 100/year:34%; 50-99:36%; 1-50:30%); 2 programs reported no TAVR in 2018. TAVR coordinators described peri-procedural code status policies and practices and how they accommodate patients' goals of care. We used data from the Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapy Registry, stratified by programs' DNR practice, to examine differences in program size, patient characteristics and risk status, and outcomes.Nearly all TAVR programs (48/50: 96%) addressed peri-procedural code status, yet only 26% had established policies. Temporarily rescinding DNR status until after TAVR was the norm (78%), yet time frames for reinstatement varied (38%48 h post-TAVR; 44% 48 h-to-discharge; 18%30 days post-discharge). For patients with fluctuating code status, no routine practices for discharge documentation were well-described. No clinically substantial differences by code status practice were noted in Society of Thoracic Surgeons Predicted Risk of Mortality risk score, peri-procedural or in-hospital cardiac arrest, or hospice disposition. Six programs maintaining DNR status recognized TAVR as a palliative procedure. Among programs categorically reversing patients' DNR status, the rationale for differing lengths of time to reinstatement reflect divergent views on accountability and reporting requirements.Marked heterogeneity exists in management of peri-procedural code status across TAVR programs, including timeframe for reestablishing DNR status post-procedure. These findings call for standardization of DNR decisions at specific care points (before/during/after TAVR) to ensure consistent alignment with patients' health-related goals and values.

Palliative care has potential to improve quality of life and goal-concordant care for patients with adult congenital heart disease (ACHD). However, it is rarely employed prior to critical illness because the best methods for implementation are not well-defined. We qualitatively evaluated ACHD patients' understanding of and opinions regarding palliative care and advance care planning (ACP) to better define the needs of this population. We conducted a thematic analysis of 25 semistructured interviews with patients with ACHD in which we assessed participants' perspectives on the need for, and barriers and facilitators to, the use of palliative care and ACP. In a group of participants with ACHD (mean age 38, 48% male) classified as simple (24%), moderate (32%), or complex (44%), we identified 4 major themes: (1) using knowledge to combat future uncertainties; (2) unfamiliarity with and limited exposure to palliative care and ACP; (3) facilitators and barriers to engaging in palliative care and ACP; and (4) importance of timing and presentation of ACP discussions. In conclusion, participants expressed a desire for knowledge about ACHD progression and treatment. They supported routine incorporation of palliative care and ACP and identified related facilitators and barriers to doing so. Importantly, timing and format of these discussions must be individualized using shared decision-making between clinicians, patients, and their families.

Learning objectives Cardiovascular disease remains a leading cause of death internationally despite decades of advances in medical, surgical and device therapy. Our armament of treatments may support heart function, correct valvular dysfunction, ameliorate arrhythmias and delay death while improving functional status. However, none of these treatments are curative, and each carries at least a small risk of unintended negative consequences, including causing or prolonging suffering. Cardiovascular disease manifests in a variety of clinical profiles, ranging from a previously healthy person with an acute myocardial infarction to a middle-aged individual with an inflammatory cardiomyopathy to a teen with a Fontan repair of single ventricle disease. Their disease experiences and deaths may look very different from one another, but they can all benefit from the incorporation of palliative care. This article provides a description of palliative care as it applies to cardiology, including tools to facilitate the use of primary palliative care by cardiovascular clinicians. Palliative care is a specialised type of medical care that focuses on improving communication about goals of care (GOC), maximising quality of life and reducing symptoms.1 2 It is patient-centred and family centred care that is appropriate at any age and at any stage of a serious illness. In the past, it was often used synonymously with hospice, particularly in end-stage cancer, and gained a reputation as ‘doing nothing’ or ‘giving up’. However, palliative care is better seen as an ‘interventional’ specialty because of its focus on GOC that frame the appropriateness of therapies and its use of measures to ameliorate suffering. Palliative care should be applied alongside usual care in acute and chronic …

Background:Advance care planning and palliative care are gaining recognition as critical care components for adults with CHD, yet these often do not occur. Study objectives were to evaluate ACHD providers’ 1) comfort managing patients’ physical symptoms and psychosocial needs and 2) perspectives on the decision/timing of advance care planning initiation and palliative care referral.Methods:Cross-sectional study of ACHD providers. Six hypothetical patients were described in case format, followed by questions regarding provider comfort managing symptoms, initiating advance care planning, and palliative care referral.Results:Fifty providers (72% physicians) completed surveys. Participants reported low levels of personal palliative care knowledge, without variation by gender, years in practice, or prior palliative care training. Providers appeared more comfortable managing physical symptoms and discussing prognosis than addressing psychosocial needs. Providers recognised advance directives as important, although the percentage who would initiate advance care planning ranged from 18 to 67% and referral to palliative care from 14 to 32%. Barriers and facilitators to discussing advance care planning with patients were identified. Over 20% indicated that advance care planning and end-of-life discussions are best initiated with the development of at least one life-threatening complication/hospitalisation.Conclusions:Providers noted high value in advance directives yet were themselves less likely to initiate advance care planning or refer to palliative care. This raises the critical questions of when, how, and by whom discussion of these important matters should be initiated and how best to support ACHD providers in these endeavours.

The COVID-19 pandemic poses risk for worsened quality of life in patients with adult congenital heart disease. In a qualitative thematic analysis of semi-structured interviews conducted July 2020 to August 2021, we examined the pandemic’s impact on participants’ (N = 25) experiences with self-perception and coping. All had moderate or complex disease; median age 32 years. The pandemic altered some participants’ self-perception, including increased vulnerability beyond heart-attributed risk. Restrictions frequently prevented participants from using their usual coping strategies, forcing use of alternative methods. For an already at-risk population, these findings suggest the need for increased mental health awareness, assessment, and support.

STRUCTURED Abstract Background As clinical care and research in adult congenital heart disease (ACHD) increasingly include a focus on quality of life, hearing and reflecting on our patients’ experiences must be a priority. The objective of this study was to describe how living with this chronic illness has affected the adult lives of patients living with ACHD. Methods We conducted a qualitative study of 25 participants in which we asked about the impact of ACHD on their quality of life. We used thematic analysis to analyze the interview data and identified themes reflecting patients’ reported experiences. Results Study participants were a mean age of 38 years (range 21–63 years) and reported good functional status. ACHD was characterized as simple (24%), moderate (32%), or complex (44%). Three major themes arose: 1) impact of ACHD on identity, including perception of “normal,” self-esteem, and motivation; 2) sources of emotional distress; and 3) influence on adult decision-making, including careers and relationships. Conclusion Living with ACHD impacts important aspects related to self-perceived quality of life. Addressing these concerns is important to providing comprehensive, patient-centered ACHD care.

Context Little is known about advance care planning (ACP) and palliative care needs among adults with congenital heart disease (ACHD). Objectives The objective of this study was to identify and synthesize studies concerning palliative care among ACHD patients. Methods We searched five electronic databases (PubMed, Embase, SCOPUS, Web of Science, and CINAHL) using the keywords palliative care and congenital heart disease. Inclusion criteria were adults (age > 18 years) with congenital heart disease and publications in English through March 3, 2019. Results Our search yielded 2872 studies, and after removal of duplicates, we screened 2319 abstracts and identified seven for inclusion. Study findings were grouped into three domains: ACP, symptomatology, and end-of-life care. Among the five cross-sectional studies, only 1%–28% of ACHD patients recalled participating in ACP discussions with their doctors but 69%–78% reported a strong interest and desire to participate in ACP. In one study, 46% (n = 67) of patients had elevated anxiety symptoms (Hospital Anxiety and Depression Scale [HADS-A] ≥ 8) and 11% (n = 15) had elevated depressive symptoms (HADS-A ≥ 8). ACHD patients who had a documented goals of care conversation before cardiac decompensation had a lower incidence of resuscitation and aggressive treatments at end of life (12% [n = 3] vs. 100% [n = 12], P Conclusion While few ACHD patients complete advance directives, our findings support that many ACHD patients recognize the value of initiating end-of-life and goals of care conversations early on in the course of illness. Future studies investigating communication and implementation strategies of ACP as well as the symptom experience of patients with ACHD are needed.

Background Patients with adult congenital heart disease (ACHD) report that advance care planning (ACP) is important, and that they want information about prognosis. However, recognizing importance and being willing to participate are different constructs, and how and when to begin ACP and palliative care discussions remains ill-defined. Methods We conducted a cross-sectional survey of 150 consecutive outpatients to assess willingness to participate in ACP, with whom, and important barriers and facilitators to these discussions. Results The majority of participants (69%) reported being willing to participate in ACP; 79% to have a meeting to discuss goals and care preferences; and 91% to speak to a clinician who specializes in palliative care. Being married and anticipating a shorter lifespan were associated with increased reported willingness to participate in ACP. The health care provider with whom most participants preferred to have these discussions was their ACHD clinician. Participants identified important barriers and facilitators to these discussions. Conclusion Patients with ACHD report being willing to participate in ACP and palliative care discussions. Patients prefer to have these discussions with their ACHD clinicians, thus ACHD clinicians need to be prepared to address these issues as part of routine care.

A 68-year-old man with ischaemic cardiomyopathy presents with acute on chronic systolic heart failure with severe mitral regurgitation. He has a history of coronary artery disease status post-coronary artery bypass surgery, stage 3 chronic kidney disease and diabetes. He is deemed high risk for surgical valve replacement and is being evaluated for transcatheter mitral valve intervention. In a goals-of-care discussion, he expresses that it is important to him to be functional and independent in his daily activities, and he wishes never to be placed on dialysis. His independence is more important to him than longevity at all costs. He is unsure how he feels about mitral valve intervention. His son, who is his healthcare power of attorney, is in favour of the intervention because he wants his father to live as long as possible, even if he is not as independent as he would like to be. They ask you how they should proceed. Cardiovascular disease is a chronic illness with periods of acute exacerbation and intense decision-making. Our therapies are not curative, and they carry the potential to be burdensome to the patient if used unwisely. Palliative care (PC) is an approach to medical care that focuses on managing symptoms, providing emotional and spiritual support, promoting communication …

Context Women with adult congenital heart disease (ACHD) have an increased risk of adverse events during pregnancy. Advance care planning may therefore be an appropriate component of prenatal care. Objective The aim of this study was to describe the perspectives of women with ACHD surrounding advance care planning during pregnancy. Methods We conducted a thematic analysis of 25 semi-structured interviews with women with ACHD who had been pregnant. Purposive sampling was used to gain diversity in ACHD lesion complexity, race, age at pregnancy, and marital status. Results Mean age at pregnancy was 29 years (range 15–41 years), and ACHD was classified as simple (24%), moderate (44%), or complex (32%). We identified three primary themes: 1) the role of advance care planning in being prepared and providing security for family; 2) reasons for avoiding advance care planning, including its lower priority among more pressing concerns and the impact it might have on their current psychological state; and 3) varied openness to advance care planning discussions during pregnancy. Conclusion Advance care planning is not a routine part of prenatal care in ACHD, and its role in this population requires further assessment.

In the current era, most individuals born with congenital heart disease will reach adulthood. There are now more adults than children living with congenital heart disease in developed countries, and the population continues to expand. Although the life-saving accomplishments achieved in pediatric cardiology are to be extolled, longer-term sequelae also warrant close attention. Adults with moderate or complex disease continue to face cardiac and noncardiac comorbidities as well as a shorted life expectancy. This article begins with a summary of common comorbidities and patterns of mortality faced by adults with congenital heart disease. This is followed by an overview of the broader impact of these issues on the lives of patients, as well as a call for interdisciplinary and comprehensive care to optimize patient outcomes.

A 63-year-old man with end-stage ischemic cardiomyopathy presented with incessant ventricular tachycardia. He had been hospitalized multiple times in the past year for severe heart failure. As he approached end of life and was regularly receiving defibrillator shocks, his care team recommended deactivation of his implantable cardioverter-defibrillator. However, his family did not wish to allow deactivation, reporting a religious obligation to prolong his life, regardless of the risk of suffering. The patient was unable to adequately participate in the decision-making process. An implantable cardioverter-defibrillator can serve to avoid sudden death but may lead to a prolonged death from heart failure. This possibility forces the examination of values regarding prolongation of life, sometimes producing disagreement among stakeholders. Although ethical consensus holds that defibrillator deactivation is legal and ethical, disagreements about life prolongation may complicate decision making. The ethical, technical, and medical complexity involved in this case speaks to the need for clear, prospective communication involving the patient, the patient's family, and members of the care team.

Recommendations for advance care planning in adults with congenital heart disease were recently published, reinforcing the role of advance care planning as an essential component of patient-centered care. In significant ways, the adult congenital heart disease population differs from the “typical” elderly population about whom much of the palliative care literature is written. Herein we report the results of a multidisciplinary conference focusing on the unique nature of this population and their needs for advance care planning. First, we describe the characteristics of adults with congenital heart disease that set them apart, including childhood illness and early interactions with the healthcare system. Then we give tailored recommendations to providers for how to effectively elicit their patients’ goals and values, in order to counsel them about the future.

Background Heart failure is the leading cause of morbidity and mortality for adults with congenital heart disease (ACHD). Many patients are ineligible for transplantation, and those who are eligible often face long wait times with high wait-list morbidity. Durable mechanical circulatory support (MCS) may be an option for many patients. This systematic review evaluates the published literature on the use of durable MCS in teenagers and adults with congenital heart disease. Methods A comprehensive search of MEDLINE (PubMed), EMBASE, and the Cochrane Library was performed electronically in July 2015 and updated in March 2016, guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. Results Individual case reports and several case series identified 66 patients with ACHD treated with durable MCS. More than half were INTERMACS 1 or 2 at the time of implantation. Patients with Fontan repairs were more frequently classified as INTERMACS 1 or 2 (89% compared to 59% or less among other groups). Cases published after 2010 showed a trend toward less severe INTERMACS status, and patients were less likely to have received transplants by the time of reporting (31% compared to 61% prior). Durable MCS was implanted as bridge-to-transplant in 77%. Patients with Fontan repair accounted for 14% of cases. Conclusion Reports of durable MCS utilization in patients with ACHD are becoming more frequent and devices are being implanted in more stable patients. Reports are mostly case reports or small case series so reporting bias is likely and prospective protocoled reporting is needed.

Introduction There is relatively sparse literature on the use of administrative datasets for research in patients with adult congenital heart disease (ACHD). The goal of this analysis is to examine the accuracy of administrative data for identifying patients with ACHD who died. Methods A list of the International Classification of Diseases codes representing ACHD of moderate- or great-complexity was created. A search for these codes in the electronic health record of adults who received care in 2010-2016 was performed, and used state death records to identify patients who died during this period. Manual record review was completed to evaluate performance of this search strategy. Identified patients were also compared with a list of patients with moderate- or great-complexity ACHD known to have died. Results About 134 patients were identified, of which 72 had moderate- or great-complexity ACHD confirmed by manual review, yielding a positive predictive value of 0.54 (95% CI 0.45, 0.62). Twenty six patients had a mild ACHD diagnosis. Thirty six patients had no identified ACHD on record review. Misidentifications were attributed to coding error for 19 patients (53%), and to acquired ventricular septal defects for 11 patients (31%). Diagnostic codes incorrect more than 50% of the time were those for congenitally corrected transposition, endocardial cushion defect, and hypoplastic left heart syndrome. Only 1 of 21 patients known to have died was not identified by the search, yielding a sensitivity of 0.95 (0.76, 0.99). Conclusion Use of administrative data to identify patients with ACHD of moderate or great complexity who have died had good sensitivity but suboptimal positive predictive value. Strategies to improve accuracy are needed. Administrative data is not ideal for identification of patients in this group, and manual record review is necessary to confirm these diagnoses.

OBJECTIVE Overall health care resource utilization by adults with congenital heart disease has increased dramatically in the past two decades, yet little is known about utilization patterns at the end of life. The objective of this study is to better understand the patterns and influences on end-of-life care intensity for adults with congenital heart disease. METHODS We identified a sample of adults with congenital heart disease (n = 65), cancer (n = 10 784), or heart failure (n = 3809) who died between January 2010 and December 2015, cared for in one multi-hospital health care system. We used multivariate analysis to evaluate markers of resource utilization, location of death, and documentation of advance care planning among patients with congenital heart disease versus those with cancer and those with heart failure. RESULTS Approximately 40% of adults with congenital heart disease experienced inpatient and intensive care unit (ICU) hospitalizations in the last 30 days of life; 64% died in the hospital. Compared to patients with cancer, patients with adult congenital heart disease (ACHD) were more likely to have inpatient (adjusted risk ratio 1.57; 95% CI 1.12-2.18) and ICU admissions in the last 30 days of life (adjusted risk ratio 2.56; 95% CI 1.83-3.61), more likely to die in the hospital (adjusted risk ratio 1.75; 95% CI 1.43-2.13), and more likely to have documentation of advance care planning (adjusted risk ratio 1.46; 95% CI 1.09-1.96). Compared to patients with heart failure (HF), patients with ACHD were less likely to have an ICU admission in the last 30 days of life (adjusted risk ratio 0.73; 95% CI 0.54-0.99). CONCLUSIONS Adults with congenital heart disease have significant hospital resource utilization near the end of life compared to patients with cancer, notable for more hospitalizations and a higher likelihood of death in the hospital. This population represents an important opportunity for the application of palliative and supportive care.

Valvular heart disease (VHD), particularly aortic valve disease, is prevalent with increasing incidence. When surgery is not possible, or when risks outweigh benefits, percutaneous treatment options may offer effective alternatives. However, procedures may not always go as planned, and frail patients or those whose symptoms are caused by other comorbidities may not benefit from valve intervention at all. Significant effort should be made to assess frailty, comorbidities and patient goals prior to intervention. Palliative care (PC) should play a critical role in the care of patients with severe valve disease. PC is specialised medical care that aims to optimise health-related quality of life by managing symptoms and clarifying patient values and goals of care. It should be implemented at the time of diagnosis and continue throughout the disease course. Because of the paucity of studies dedicated to the provision of PC to patients with advanced VHD, further research is needed.

Introduction: With improved management, there are now more adults with congenital heart disease (ACHD) than children. However, long-term survival with moderate or severe ACHD remains limited, and there is relatively sparse literature on the intensity or quality of end-of-life care for these patients. The goal of this analysis is to examine the accuracy of administrative data for identifying patients who died with ACHD to facilitate study of care provided near the end of life. Methods: We created a list of ICD-9 and ICD-10 codes representing ACHD of moderate or great complexity. We performed a search for these codes in the electronic health record (EHR) of adults who received care 2010-2016 within our healthcare system. We used state death records to identify which of these patients died during the same timeframe. Manual EHR review was completed to evaluate performance of this search strategy. Identified patients were also compared to a list of patients seen in our ACHD clinic and known to have died during 2010-2016. Results: Using ICD data, 121 patients were identified, of which 66 actually had the moderate or greater complexity ACHD conditions by EHR review (positive predictive value, 0.55; 95% confidence interval 0.45, 0.63). EHR review confirmed 12 patients with Eisenmenger Syndrome, for which there is no specific ICD code. “Cyanosis+other” did not identify any of these, “VSD+other” (ventricular septal defect) identified 6, and there were 6 whose only ACHD code was VSD. Of the remaining 55 patients, 24 had ACHD not on the targeted list, largely due to coding error. In addition, despite being coded as having ACHD, 31 patients had no identified ACHD on EHR review. These misidentifications were attributed to coding error for 15 patients. Another 11 patients (35%) had acquired VSD due to myocardial infarction or endocarditis, for which there is no separate ICD code. Codes with the highest degree of error, incorrect more than 50% of the time, were those for congenitally corrected transposition, endocardial cushion defect, and hypoplastic left heart syndrome. The list of known deceased clinic patients included 21 with ACHD of interest. Only 1 of these was not identified by the ICD search, yielding a sensitivity for our list of ICD codes in this small sample of 0.95 (0.77,0.99). Conclusion: Use of administrative data to identify patients with ACHD of moderate or great complexity who have died had good sensitivity but suboptimal positive predictive value. Strategies to improve accuracy can be employed. Excluding patients who have codes for myocardial infarction or endocarditis in addition to VSD and using “VSD+other” as an additional proxy for Eisenmenger Syndrome are two examples. Administrative data is not ideal for identification of patients with ACHD of moderate or great complexity who have died, and manual EHR review is necessary to confirm these diagnoses.

Once the focus of medical treatment transitions away from life-prolonging care, implantable cardioverter-defibrillator (ICD) shocks no longer serve their intended purpose. In this situation, device deactivation may be appropriate. The potential unwanted consequences of having an ICD at the end of life are rarely addressed, although their life-saving benefits until that point are widely recognised. In their Heart paper, Stoevelaar and colleagues1 share insight from patients’ expressions of their thoughts and experiences related to advance care planning surrounding ICD deactivation. Five focus groups were held with 41 participants, exploring patient attitudes towards ICD deactivation and delivery of information and communication about ICDs towards the end of life. Using thematic analysis, the authors present results centred about three major themes: (1) reflection on wishes and preferences, (2) discussing preferences, and (3) recording and reviewing preferences. Participants had widely differing opinions about ICD deactivation, ranging from equating deactivation with euthanasia to fearing shocks more than death. One was even unaware of the option to deactivate their ICD. Living with compromised quality of life was deemed undesirable, and several participants cited suffering from non-cardiac disease as a reason to deactivate the ICD. Others felt no need to even think about future ICD deactivation, instead preferring to focus on life. While some participants desired conversations about ICD deactivation with healthcare providers, few had done so, and this served as a source of discontent for some. Strikingly, there was no consensus as to the optimal timing for …

Heterotaxy syndrome occurs in 1:10,000 birth. It is characterized by absence of left-right asymmetry of the thoracoabdominal organs. Associated cardiovascular anomalies are common. Cardiovascular procedures in these patients can be challenging due to difficult vascular accesses into the heart. 41

J Clin Hypertens (Greenwich). 2012;14:711–717. ©2012 Wiley Periodicals, Inc. One quarter of Americans will be older than 80 by 2030, and hypertension in this group will be ubiquitous. Most studies of hypertension exclude octogenarians and are not able to be generalized because of differences in physiologic and social phenomena. This review evaluates the existing literature in an effort to identify unresolved issues and guide future areas of investigation. Hypertension has been well characterized in adults and the young elderly, and the focus is on optimizing treatment regimens. However, research specifically involving this rapidly growing minority is sparse. Most studies are still trying to characterize blood pressure goals, and only one major series has begun to evaluate treatment options. Based on available evidence, it appears that an appropriate blood pressure goal may be somewhat higher in octogenarians and that thiazide diuretics may be the initial treatment of choice.

The diagnosis of constrictive pericarditis (CP) often is elusive due to varied causes with nonspecific clinical findings. Conservative management is difficult and surgical pericardiectomy is sometimes required for diagnosis, as well as therapy. A 78-year-old man presented with dyspnea, peripheral

Introduction The intra-aortic balloon pump (IABP) is the most frequently utilized form of temporary mechanical circulatory support (MCS) in cardiogenic shock (CS). Withdrawal of IABP support may precipitate hemodynamic compromise such that IABP reinsertion is required. Data are scarce regarding the incidence and outcomes of patients undergoing IABP reinsertion in this setting. Methods In this single-center retrospective study, we identified consecutive patients with CS in whom IABP reinsertion was required for hemodynamic decompensation. These patients were compared to matched controls in whom IABP withdrawal was successful. The primary outcome measure was in-hospital mortality, while the secondary outcome measure was a composite of in-hospital death, need for advanced MCS or heart transplantation, or discharge to hospice. Results Among 222 patients requiring IABP for CS, we identified 20 case patients (incidence = 9.0%) and 38 matched controls. Baseline characteristics were similar for the two groups. In-hospital mortality was 70% in the reinsertion group and 31% in the controls (Odds ratio (OR) 5.2, 95% CI 1.4–18.9, P = 0.005). The composite secondary endpoint was also significantly more common in the reinsertion group than the controls (85% vs. 42%; OR 7.3, 95% CI 1.6–33.1, P = 0.002). On multivariate analysis, the need for IABP reinsertion was independently associated with in-hospital mortality (OR 7.7, 95% CI 1.6–36.2, P = 0.01). Conclusion Among patients with CS undergoing IABP removal, hemodynamic deterioration requiring IABP reinsertion is associated with extremely poor outcomes and, in appropriate patients, should prompt consideration of more advanced cardiac support.

Despite widespread availability of imaging modalities, there exists a paucity of data to support recommendations surrounding their use. The development of appropriate use criteria (AUC) targets this gap by addressing how to decide whether a test is a reasonable choice for a particular patient. Generated by a rigorous process modeled from the Rand methodology, AUC for cardiac computed tomography were initially developed in 2006 to guide optimal patient selection, and they were revised in 2010 in an effort to guide appropriate utilization of testing. The 2010 revision rated 93 clinical scenarios, expanded from the original set of 37 scenarios, with a general increase in the proportion of appropriate clinical scenarios. Strengths of cardiac computed tomography, as reflected in the proportion of scenarios rated as appropriate, include use in chest pain and for evaluation of cardiac structure and function. Recent data suggest that education and an emphasis on AUC can improve performance in patient selection.