Transcatheter Interventions in Adult Congenital Heart Disease.

Congenital heart disease (CHD) is the most common congenital birth defect with an incidence of 1 in 100. Current survival to adulthood is expected in 9 out of 10 children with severe CHD as the diagnostic, interventional, and surgical success improves. The adult CHD (ACHD) population is increasingly diverse, reflecting the broad spectrum of CHD and evolution of surgical techniques to improve survival. Similarly, transcatheter interventions have seen exponential growth and creativity to reduce the need for repeat sternotomies. This article focuses on newer data and evolving techniques for transcatheter interventions specific to certain ACHD populations.

A unique cause of coronary obstruction after transcatheter aortic valve replacement.

Delayed coronary obstruction is a rare but often fatal complication of transcatheter aortic valve replacement that can present within 24 hr after the transcatheter aortic valve replacement procedure. We present a case of a 77-year-old woman whose distal left main coronary artery bifurcation became obstructed by an embolized piece of native valve leaflet material 16 hours after the uncomplicated transfemoral implantation of an Edwards Sapien Ultra 23 mm aortic valve, which was successfully treated with the simultaneous kissing stent technique.

Validity of echocardiography for detection of left ventricular thrombus with surgical validation in patients awaiting durable left ventricular assist device.

OBJECTIVE: Unrecognized left ventricular thrombi (LVT) can have devastating clinical implications and precludes patients with end-stage heart failure from undergoing left ventricular assist device (LVAD) implantation without cardiopulmonary bypass assistance. We assessed the reliability of an echocardiogram to diagnose LVT in patients with end-stage heart disease who underwent LVAD implantation. METHODS: A single-center retrospective study evaluated 232 consecutive adult patients requiring implantation of durable LVADs between 2005 and 2019. The validity of preoperative transthoracic echocardiogram (TTE) and intraoperative transesophageal echocardiogram (TEE) for diagnosing LVT was compared to direct inspection at the time of LVAD implantation. RESULTS: There were 232 patients that underwent LVAD implantation, with 226 patients (97%) receiving a preoperative TTE. Of those 226 patients, 32 patients (14%) received ultrasound enhancing agents (UEA). Intraoperative TEE images were available in 195 patients (84%). The sensitivity of TTE without UEA was 22% and specificity was 90% for detecting LVT, compared to 50% and 86%, respectively, for TTE with UEA. For intraoperative TEE, the sensitivity and specificity were 46% and 96%, respectively. The false omission rate ranged from 4% to 8% for all modalities of echocardiography. CONCLUSION: Among patients undergoing LVAD implantation, preoperative TTE and intraoperative TEE had poor sensitivity for LVT detection. Up to 8% of echocardiograms were incorrectly concluded to be negative for LVT on surgical validation. The low sensitivity and positive predictive value for diagnosing LVT suggest that echocardiography has limited reliability in this cohort of patients who are at high risk of LVT formation and its subsequent complications.

Impact of Sex on Outcomes Among Patients With Cardiac Allograft Vasculopathy Who Undergo Percutaneous Coronary Intervention.

BACKGROUND: Orthotopic heart transplantation (OHT) is a reasonable treatment strategy for patients with endstage heart failure. Percutaneous coronary intervention (PCI) can be performed to treat cardiac allograft vasculopathy (CAV). We sought to examine sex-based differences and outcomes in patients undergoing PCI for CAV. METHODS: This was a single- center, retrospective study of consecutive patients undergoing PCI for CAV from July 1993 to July 2017. The primary outcome was a composite of death, myocardial infarction (MI), target-vessel revascularization (TVR), or repeat OHT. RESULTS: A total of 140 patients (39% women) who underwent PCI for CAV were studied over a median follow-up of 3.9 years. Women were more likely to be younger and had less hypertension, with a trend toward less diabetes and higher ejection fractions. No significant differences in the primary composite outcome were noted between women and men (hazard ratio, 1.16; 95% confidence interval, 0.76-1.76; P=.49), as well as the individual outcomes of death, MI, or TVR (P>.05 for all). Women were more likely to undergo repeat OHT than men (hazard ratio, 2.93; 95% confidence interval, 1.48-5.81; P<.01). After covariate adjustment, female sex and younger age were independently associated with repeat OHT for CAV (P<.05). No differences in the primary outcome as well as the individual outcomes of death, MI, or TVR were noted between sex groups in patients who underwent PCI for CAV. CONCLUSION: Female patients were more likely to undergo redo OHT for CAV. Younger age at time of initial OHT was associated with a need for repeat OHT.

Risk factors for infective endocarditis following transcatheter pulmonary valve replacement in patients with congenital heart disease.

OBJECTIVES: We sought to delineate the risk factors for infective endocarditis (IE) in patients undergoing transcatheter pulmonary valve replacement (TCPVR). BACKGROUND: Despite the therapeutic benefits of TCPVR for treatment of dysfunctional right ventricular outflow tracts, IE is a major complication of the approach. Specific hemodynamic gradients and patient immune status as predisposing factors for IE are largely unexplored. METHODS: We performed a retrospective review of patients who had undergone TCPVR at UCLA between October 2010 and October 2017. Cases of IE were diagnosed based on the modified Duke criteria. RESULTS: Two hundred and thirty-five cases of TCPVR were performed with a mean follow-up of 2.6 years (range 0.0-8.0 years). Sixteen distinct IE events developed in 13 patients (Melody™ n = 12, SAPIEN n = 1), with a median time from implant to IE of 3.3 years (range 2.0-7.2 years). Univariate Cox regression showed that immunocompromised status was significantly associated with the development of IE hazard ratios (HR 5.43 [1.80-16.4], p = .003). Kaplan-Meier curves show that the 5-year freedom from IE among immunocompetent patients was 87% (95% CI 78-96%) versus 64% (95% CI 39-89%) among immunocompromised patients (log-rank p = .02). Postimplant right ventricular systolic pressure was higher among immunocompromised patients (p = .03). The risk of IE post-TCPVR in immunocompromised patients with residual pulmonary stenosis was 43%. CONCLUSIONS: Among the risk factors examined in this study, immunocompromised status was the most significant predictor of IE development post-TCPVR. Patients with the lowest risk of IE are those with competent immune systems, without a history of IE, and with minimal residual pulmonary valve gradients post-TCPVR.

Postoperative and short-term atrial tachyarrhythmia burdens after transcatheter vs surgical pulmonary valve replacement among congenital heart disease patients.

OBJECTIVE: We examined the atrial tachyarrhythmia (AT) burden among patients with congenital heart disease (CHD) following transcatheter (TC-) or surgical (S-) pulmonary valve replacement (PVR). DESIGN/SETTING: This was a retrospective observational study of patients who underwent PVR from 2010 to 2016 at UCLA Medical Center. PATIENTS: Patients of all ages who had prior surgical repair for CHD were included. Patients with a history of congenitally corrected transposition of the great arteries, underwent a hybrid PVR procedure, or had permanent atrial fibrillation (AF) without a concomitant ablation were excluded. OUTCOME MEASURES: The primary outcome was a time-to-event analysis of sustained AT. Sustained ATs were defined as focal AT, intra-atrial reentrant tachycardia/atrial flutter, or AF lasting at least 30 seconds or terminating with cardioversion or antitachycardia pacing. RESULTS: Two hundred ninety-seven patients (TC-PVR, n = 168 and S-PVR, n = 129) were included. During a median follow-up of 1.2 years, nine events occurred in TC-PVR group (5%) vs 23 events in S-PVR group (18%). In the propensity adjusted models, the following factors were associated with significant risk of AT after PVR: history of AT, age at valve implantation, severe right atrial enlargement, and S-PVR. In the secondary analysis, TC-PVR was associated with lower adjusted risk of AT events in the postoperative epoch (first 30 days), adjusted IRR 0.31 (0.14-0.97), P = .03, but similar risk in the short-term epoch, adjusted IRR 0.64 (0.14-2.94), P = .57. CONCLUSION: There was an increased risk of AT in the first 30 days following S-PVR compared to TC-PVR. Additional factors associated with risk of AT events after PVR were a history of AT, age at valve implantation, and severe right atrial enlargement.

Ventricular arrhythmia burden after transcatheter versus surgical pulmonary valve replacement.

OBJECTIVE: Comparative ventricular arrhythmia (VA) outcomes following transcatheter (TC-PVR) or surgical pulmonary valve replacement (S-PVR) have not been evaluated. We sought to compare differences in VAs among patients with congenital heart disease (CHD) following TC-PVR or S-PVR. METHODS: Patients with repaired CHD who underwent TC-PVR or S-PVR at the UCLA Medical Center from 2010 to 2016 were analysed retrospectively. Patients who underwent hybrid TC-PVR or had a diagnosis of congenitally corrected transposition of the great arteries were excluded. Patients were screened for a composite of non-intraoperative VA (the primary outcome variable), defined as symptomatic/recurrent non-sustained ventricular tachycardia (VT) requiring therapy, sustained VT or ventricular fibrillation. VA epochs were classified as 0-1 month (short-term), 1-12 months (mid-term) and ≥1 year (late-term). RESULTS: Three hundred and two patients (TC-PVR, n=172 and S-PVR, n=130) were included. TC-PVR relative to S-PVR was associated with fewer clinically significant VAs in the first 30 days after valve implant (adjusted HR 0.20, p=0.002), but similar mid-term and late-term risks (adjusted HR 0.72, p=0.62 and adjusted HR 0.47, p=0.26, respectively). In propensity-adjusted models, S-PVR, patient age at PVR and native right ventricular outflow tract (RVOT) (vs bioprosthetic/conduit outflow tract) were independent predictors of early VA after pulmonary valve implantation (p<0.05 for all). CONCLUSION: Compared with S-PVR, TC-PVR was associated with reduced short-term but comparable mid-term and late-term VA burdens. Risk factors for VA after PVR included a surgical approach, valve implantation into a native RVOT and older age at PVR.

Transcatheter interventions in adults with congenital heart disease: Surveys from the Society for Cardiovascular Angiography and Interventions to identify current patterns of care and perception on training requirements.

BACKGROUND: Interventional catheterization is central to the care of Adults with Congenital Heart Disease (ACHD). Current standards for care provision and training in ACHD intervention are lacking. We sought to examine trends in current practice and training among interventionalists. METHODS: We analyzed the results of two separate international surveys in June 2016. One was sent to all active members from the Society of Cardiovascular Angiography and Interventions (SCAI) who self-identified Structural Heart Disease or Congenital Heart Disease as a practice area. The second survey was conducted through the Pediatric Interventional Early Career Society (PICES) aimed at pediatric and adult congenital interventionalists in their first seven years after training. The total survey sample sizes were 1,535 and 112, respectively. RESULTS: Response rates for the SCAI and PICES surveys were 15% (237/1,535) and 75% (84/112), respectively. Most respondents (74%) worked at institutions with pediatric and adult facilities in proximity (major medical centers). While 75% of SCAI respondents believed complex transcatheter procedures should be performed by ACHD-trained interventionalists or multidisciplinary teams, only 32% reported such care is being provided at the present time. Most pediatric and adult cardiologists surveyed (73%) do not believe current interventional fellowships provide adequate training for proficiency in ACHD interventions. CONCLUSIONS: ACHD management remains underdeveloped in relative terms, particularly in the United States. Significant gaps in interventional standards of practice and future training needs were recognized by this study. Our survey should serve as an impetus to establish training guidelines for physicians who seek to perform ACHD interventions.

In-hospital statin underutilization among high-risk patients: delayed uptake of the 2013 cholesterol guidelines in a U.S. cohort.

OBJECTIVES: Clinician utilization of the 2013 cholesterol lowering guidelines remains variable and unknown. We sought to examine statin prescribing patterns and compare rates among specialists who treat high-risk cardiovascular patients admitted to the hospital. METHODS: We retrospectively (via chart review) examined four specialty groups: (i) Cardiology, (ii) Cardiovascular or Vascular (CV) Surgery, (iii) Neurology, and (iv) Internal Medicine. Adult patients were included based on a discharge diagnosis of acute coronary syndrome, coronary artery bypass graft surgery, carotid endarterectomy, acute ischemic stroke, transient ischemic attack, or high-risk chest pain. Prescribing patterns were evaluated 6 months and 18 months after the release of the 2013 guidelines. High-intensity statin was defined as atorvastatin 40-80 mg or rosuvastatin 20-40 mg per day. RESULTS: 632 patients were included in our study. The following percentages of patients were discharged on high-intensity statin (6 months; 18 months): (i) Cardiology (80%; 85%), (ii) CV Surgery (52%, 65%), (iii) Neurology (59%; 66%), and (iv) Internal Medicine (45%; 48%). Among the four groups, Cardiology was the most likely to discharge patients on high-intensity statin (p < 0.001) in 2014 and in 2015. Cardiology, CV Surgery, and Neurology significantly increased the percentage of patients on high-intensity statin from pre-admission to time of discharge in both years. CONCLUSION: High-intensity statin therapy is underutilized among high-risk cardiovascular patients admitted to the hospital. Variations exist in prescribing patterns of different specialties who manage high-risk populations. This data can be used to test quality improvement interventions to improve rates of high-intensity statin utilization among high-risk patients prior to hospital discharge.

Early detection of right ventricular dysfunction using transthoracic echocardiography in ARDS: a more objective approach.

PURPOSE: Right ventricular (RV) dysfunction is an independent predictor of morbidity and mortality in acute respiratory distress syndrome (ARDS). Our goal was to describe morphologic changes in the RV using objective measures on transthoracic echocardiography (TTE) that occur following ARDS. METHODS: We retrospectively measured changes in the following RV parameters from a pre-ARDS TTE to an ARDS TTE: tricuspid annular plane systolic excursion (TAPSE), myocardial performance index (MPI), fractional area change (FAC), systolic pulmonary artery pressure (SPAP), peak tricuspid regurgitant (TR) velocity, and septal shift. RESULTS: Over 24 months, 14 patients met inclusion/exclusion criteria. Mean TAPSE decreased from 22.4 mm pre-ARDS to 16.3 mm during ARDS, P<.001. Mean MPI increased from 0.19 to 0.38, P=.001. Mean FAC decreased from 60.8% to 41.2%, P=.003. Peak TR velocity increased from 2.67 m/s pre-ARDS to 3.31 m/s during ARDS, P=.02. SPAP and septal shift demonstrated trends but not statistically different between pre-ARDS and ARDS states. TAPSE correlated with ARDS severity (PaO2 /FiO2 ratios), P=.004, and was lower among 30-day nonsurvivors compared with survivors, P=.002. CONCLUSIONS: Mild RV dysfunction is common after ARDS onset. RV morphologic changes coupled with dysfunction can be detected noninvasively through TTE changes with TAPSE, MPI, and FAC. Mild RV dysfunction by TAPSE is associated with ARDS severity and mortality.

Echocardiographic parameters of right ventricular function predict mortality in acute respiratory distress syndrome: a pilot study.

Right ventricular (RV) dysfunction in acute respiratory distress syndrome (ARDS) contributes to increased mortality. Our aim is to identify reproducible transthoracic echocardiography (TTE) parameters of RV dysfunction that can be used to predict outcomes in ARDS. We performed a retrospective single-center cohort pilot study measuring tricuspid annular plane systolic excursion (TAPSE), Tei index, RV-fractional area change (RV-FAC), pulmonary artery systolic pressure (PASP), and septal shift, reevaluated by an independent blinded cardiologist (JK). Thirty-eight patients were included. Patients were divided on the basis of 30-day survival. Thirty-day mortality was 47%. Survivors were younger than nonsurvivors. Survivors had a higher pH, PaO2∶FiO2 ratio, and TAPSE. Acute Physiology and Chronic Health Evaluation II (APACHE II), Simplified Acute Physiology Score II (SAPS II), and Sequential Organ Failure Assessment (SOFA) scores were lower in survivors. TAPSE has the strongest association with increased 30-day mortality from date of TTE. Accordingly, TAPSE has a strong positive correlation with PaO2∶FiO2 ratios, and Tei index has a strong negative correlation with PaO2∶FiO2 ratios. Septal shift was associated with lower PaO2∶FiO2 ratios. Decrease in TAPSE, increase in Tei index, and septal shift were seen in the severe ARDS group. In multivariate logistic regression models, TAPSE maintained a significant association with mortality independent of age, pH, PaO2∶FiO2 ratios, positive end expiratory pressure, PCO2, serum bicarbonate, plateau pressures, driving pressures, APACHE II, SAPS II, and SOFA scores. In conclusion, TAPSE and other TTE parameters should be used as novel predictive indicators for RV dysfunction in ARDS. These parameters can be used as surrogate noninvasive RV hemodynamic measurements to be manipulated to improve mortality in patients with ARDS and contributory RV dysfunction.

Contemporary Hospitalization Rate Among Adults With Complex Congenital Heart Disease.

BACKGROUND: Adults with congenital heart disease are hospitalized at increasing rates in the Western world. Identification of rates of and risk factors for hospitalization is essential for research and improving patient outcomes. METHODS: We conducted a single-center retrospective analysis of patients with a primary diagnosis of tetralogy of Fallot (TOF), transposition of the great arteries (both d- and l-transposition of the great arteries [TGAs]), or single ventricle (SV). We investigated the rates of mortality, annual hospitalization, and patient-specific risk factors for unplanned cardiac hospitalization. RESULTS: Adult patients with complex congenital heart disease are hospitalized at a rate four to eight times greater than the general US population (P < .001). In addition, there are significant differences between the rates of hospitalization in TOF and TGA (0.39 and 0.41 hospitalizations per patient-year, respectively) and SV (0.72 hospitalizations per patient-year). The majority of excess hospitalizations in the study group were due to cardiac disease (P < .001 for all three groups). Risk factors for unplanned cardiac hospitalization in TOF included pulmonary atresia, depressed left ventricular and right ventricular ejection fraction, and smoking; in TGA, they included Ebstein malformation, surgeries other than primary repair, noncardiac diagnoses, atrial arrhythmias, atrioventricular nodal block, left ventricular ejection fraction, and smoking; and in SV, they included atrial arrhythmias and cyanosis. CONCLUSIONS: Patients born with complex congenital heart disease are hospitalized far more frequently than the general US population, primarily for cardiac-related illness. Future research should focus on confirming the present findings and on identification of strategies to improve outcomes in this growing group of patients.

Platypnea-Orthodeoxia in Patients on Hemodialysis: A New Approach to Its Pathophysiology and Implications for Treatment.

Platypnea-orthodeoxia is a poorly understood clinical syndrome resulting in dyspnea and hypoxemia in the upright position, which diminishes with recumbency. Recognition of the disease is limited by its low prevalence and decreased awareness among clinicians. However, understanding the disease, its pathophysiology, its clinical presentation, and the possible therapeutic options is vital in the management of these patients. Here, we present 2 cases of platypnea- orthodeoxia where oxygen saturations worsened with hemodialysis. After highlighting the common features in the clinical pattern of each patient, we present a pressure-mediated pathophysiologic mechanism (in contrast to a previously reported morphologically based hypothesis) to explain the characteristic effects of hemodialysis on hypoxia in platypnea-orthodeoxia. We present a novel diagnostic approach using balloon occlusion testing when the diagnosis is unclear and illustrate how treatments can be tailored to the comorbidities of a specific patient.

Acute Cyclophosphamide Hemorrhagic Myopericarditis: Dilemma Case Report, Literature Review and Proposed Diagnostic Criteria.

Cyclophosphamide is a potent DNA alkylating agent used in chemotherapy and immunosuppression. Although an old agent, its use in the present day has expanded for cases of refractory autoimmune disease. In this report, a case of haemorrhagic myopericarditis resulting from high-dose cyclophosphamide for chronic inflammatory demyelinating polyneuropathy is presented. The patient had no predisposing cardiovascular risk factors and a structurally normal heart on previous echocardiogram. Following administration of high-dose cyclophosphamide, the patient developed acute congestive heart failure. Serial echocardiography demonstrated pericardial effusion, myocardial thickening, and progressive right ventricular dysfunction. Histopathology on autopsy revealed acute myocardial necrosis, intra-myocardial extravasation of blood, fibrin, and fibrin-platelet microthrombi compatible with the diagnosis of haemorrhagic myopericarditis. The ante-mortem diagnostic dilemma is described to emphasize the need for pattern recognition and clinical criteria for diagnosis. Subsequent comprehensive literature review was performed to identify features that will facilitate earlier diagnosis of haemorrhagic myopericarditis by healthcare providers.

Colistin-mediated neurotoxicity.

We describe a 51-year-old man who developed renal and neural toxicity after the administration of colistin. He developed respiratory apnoea, neuromuscular blockade and severe comatose encephalopathy with the lack of brainstem reflexes. After discontinuation of the antibiotic, he made a prompt recovery to his baseline neurological function. The case illustrates the importance of recognising the toxicities associated with colistin. Although recent literature details its nephrotoxicity, current data have been discordant with the rare cases of respiratory apnoea or neuromuscular blockade once cited over 30 years ago. Additionally, no cases have ever described the profound encephalopathy with lack of brainstem function described here. The awareness of colistin's potentially fatal effects must be kept in mind when administering this antibiotic. Vigilance of the encephalopathic picture can also facilitate the diagnosis of colistin-mediated neurotoxicity in a patient with altered mental status of otherwise unknown aetiology.