K. Hryniewicz

The 2013 International Society for Heart and Lung Transplantation Guidelines for mechanical circulatory support: Executive summary

Institutional Affiliations Co-chairs Feldman D: Minneapolis Heart Institute, Minneapolis, Minnesota, Georgia Institute of Technology and Morehouse School of Medicine; Pamboukian SV: University of Alabama at Birmingham, Birmingham, Alabama; Teuteberg JJ: University of Pittsburgh, Pittsburgh, Pennsylvania Task force chairs Birks E: University of Louisville, Louisville, Kentucky; Lietz K: Loyola University, Chicago, Maywood, Illinois; Moore SA: Massachusetts General Hospital, Boston, Massachusetts; Morgan JA: Henry Ford Hospital, Detroit, Michigan Contributing writers Arabia F: Mayo Clinic Arizona, Phoenix, Arizona; Bauman ME: University of Alberta, Alberta, Canada; Buchholz HW: University of Alberta, Stollery Children’s Hospital and Mazankowski Alberta Heart Institute, Edmonton, Alberta, Canada; Deng M: University of California at Los Angeles, Los Angeles, California; Dickstein ML: Columbia University, New York, New York; El-Banayosy A: Penn State Milton S. Hershey Medical Center, Hershey, Pennsylvania; Elliot T: Inova Fairfax, Falls Church, Virginia; Goldstein DJ: Montefiore Medical Center, New York, New York; Grady KL: Northwestern University, Chicago, Illinois; Jones K: Alfred Hospital, Melbourne, Australia; Hryniewicz K: Minneapolis Heart Institute, Minneapolis, Minnesota; John R: University of Minnesota, Minneapolis, Minnesota; Kaan A: St. Paul’s Hospital, Vancouver, British Columbia, Canada; Kusne S: Mayo Clinic Arizona, Phoenix, Arizona; Loebe M: Methodist Hospital, Houston, Texas; Massicotte P: University of Alberta, Stollery Children’s Hospital, Edmonton, Alberta, Canada; Moazami N: Minneapolis Heart Institute, Minneapolis, Minnesota; Mohacsi P: University Hospital, Bern, Switzerland; Mooney M: Sentara Norfolk, Virginia Beach, Virginia; Nelson T: Mayo Clinic Arizona, Phoenix, Arizona; Pagani F: University of Michigan, Ann Arbor, Michigan; Perry W: Integris Baptist Health Care, Oklahoma City, Oklahoma; Potapov EV: Deutsches Herzzentrum Berlin, Berlin, Germany; Rame JE: University of Pennsylvania, Philadelphia, Pennsylvania; Russell SD: Johns Hopkins, Baltimore, Maryland; Sorensen EN: University of Maryland, Baltimore, Maryland; Sun B: Minneapolis Heart Institute, Minneapolis, Minnesota; Strueber M: Hannover Medical School, Hanover, Germany Independent reviewers Mangi AA: Yale University School of Medicine, New Haven, Connecticut; Petty MG: University of Minnesota Medical Center, Fairview, Minneapolis, Minnesota; Rogers J: Duke University Medical Center, Durham, North Carolina

Advanced Heart Failure with Preserved Systolic Function in Nonobstructive Hypertrophic Cardiomyopathy: Under-Recognized Subset of Candidates for Heart Transplant

Background—In hypertrophic cardiomyopathy (HCM), heart transplant has been predominantly confined to patients with systolic dysfunction. An underappreciated HCM subset comprises patients with preserved left ventricular (LV) systolic function who may also require consideration for transplantation. Therefore, we sought to define the clinical profile and occurrence of advanced heart failure among patients with nonobstructive HCM and preserved systolic function. Methods and Results—Databases from 2 referral centers comprising 2100 HCM patients were interrogated. Forty-six nonobstructive HCM patients (2.2%) either received or were listed for heart transplant, including 20 with normal systolic function (ejection fraction ≥50%). At transplant listing, these 20 patients were 42±13 years old, each in New York Heart Association functional class III/IV with ejection fraction of 62±7%. LV was hypertrophied with maximum wall thickness of 22±4 mm and nondilated (end-diastolic dimension, 39±7 mm). Cardiovascular magnetic resonance in 10 (of 15) patients showed no or minimal fibrosis (⩽5% LV mass). Elevated LV end-diastolic or pulmonary capillary wedge pressure, consistent with diastolic dysfunction, was present in 15 patients (75%). LV filling was impaired by echocardiographic measures in all patients, including a restrictive inflow pattern in 8 (40%). In 2 patients, traditional criteria for transplant were absent, including peak VO2 >14 mL/kg/min. Heart transplantation was performed in 12 patients with each alive and without cardiovascular symptoms, 2.3±1.7 years later. Conclusions—A previously under-recognized segment of the broad HCM clinical spectrum consists of nonobstructive patients with advanced heart failure, in the presence of preserved systolic function, for whom heart transplant is the sole definitive therapeutic option.Background— In hypertrophic cardiomyopathy (HCM), heart transplant has been predominantly confined to patients with systolic dysfunction. An underappreciated HCM subset comprises patients with preserved left ventricular (LV) systolic function who may also require consideration for transplantation. Therefore, we sought to define the clinical profile and occurrence of advanced heart failure among patients with nonobstructive HCM and preserved systolic function. Methods and Results— Databases from 2 referral centers comprising 2100 HCM patients were interrogated. Forty-six nonobstructive HCM patients (2.2%) either received or were listed for heart transplant, including 20 with normal systolic function (ejection fraction ≥50%). At transplant listing, these 20 patients were 42±13 years old, each in New York Heart Association functional class III/IV with ejection fraction of 62±7%. LV was hypertrophied with maximum wall thickness of 22±4 mm and nondilated (end-diastolic dimension, 39±7 mm). Cardiovascular magnetic resonance in 10 (of 15) patients showed no or minimal fibrosis (≤5% LV mass). Elevated LV end-diastolic or pulmonary capillary wedge pressure, consistent with diastolic dysfunction, was present in 15 patients (75%). LV filling was impaired by echocardiographic measures in all patients, including a restrictive inflow pattern in 8 (40%). In 2 patients, traditional criteria for transplant were absent, including peak VO2 >14 mL/kg/min. Heart transplantation was performed in 12 patients with each alive and without cardiovascular symptoms, 2.3±1.7 years later. Conclusions— A previously under-recognized segment of the broad HCM clinical spectrum consists of nonobstructive patients with advanced heart failure, in the presence of preserved systolic function, for whom heart transplant is the sole definitive therapeutic option.

Position paper for the organization of ECMO programs for cardiac failure in adults

Extracorporeal membrane oxygenation (ECMO) has been used increasingly for both respiratory and cardiac failure in adult patients. Indications for ECMO use in cardiac failure include severe refractory cardiogenic shock, refractory ventricular arrhythmia, active cardiopulmonary resuscitation for cardiac arrest, and acute or decompensated right heart failure. Evidence is emerging to guide the use of this therapy for some of these indications, but there remains a need for additional evidence to guide best practices. As a result, the use of ECMO may vary widely across centers. The purpose of this document is to highlight key aspects of care delivery, with the goal of codifying the current use of this rapidly growing technology. A major challenge in this field is the need to emergently deploy ECMO for cardiac failure, often with limited time to assess the appropriateness of patients for the intervention. For this reason, we advocate for a multidisciplinary team of experts to guide institutional use of this therapy and the care of patients receiving it. Rigorous patient selection and careful attention to potential complications are key factors in optimizing patient outcomes. Seamless patient transport and clearly defined pathways for transition of care to centers capable of providing heart replacement therapies (e.g., durable ventricular assist device or heart transplantation) are essential to providing the highest level of care for those patients stabilized by ECMO but unable to be weaned from the device. Ultimately, concentration of the most complex care at high-volume centers with advanced cardiac capabilities may be a way to significantly improve the care of this patient population.

Acute Mechanical Circulatory Support: Bridge to Recovery or to Decision

Mechanical circulatory support (MCS) is, as the name implies, the use of electro-mechanical devices to support the circulatory system. This support may be uni-ventricular, bi-ventricular, pulmonary, percutaneous, central, or any combination of these, depending on the clinical scenario. A large component of MCS use is in a more permanent fashion in the bridge-to-transplant (BTT) or destination therapy (DT) patient. The other scenario where MCS is used is in the acute setting as a means of supporting the patient as a bridge-to-recovery or a bridge-to-decision about long-term MCS.

Advanced Heart Failure With Preserved Systolic Function in Nonobstructive Hypertrophic CardiomyopathyCLINICAL PERSPECTIVE: Under-Recognized Subset of Candidates for Heart Transplant

Background—In hypertrophic cardiomyopathy (HCM), heart transplant has been predominantly confined to patients with systolic dysfunction. An underappreciated HCM subset comprises patients with preserved left ventricular (LV) systolic function who may also require consideration for transplantation. Therefore, we sought to define the clinical profile and occurrence of advanced heart failure among patients with nonobstructive HCM and preserved systolic function. Methods and Results—Databases from 2 referral centers comprising 2100 HCM patients were interrogated. Forty-six nonobstructive HCM patients (2.2%) either received or were listed for heart transplant, including 20 with normal systolic function (ejection fraction ≥50%). At transplant listing, these 20 patients were 42±13 years old, each in New York Heart Association functional class III/IV with ejection fraction of 62±7%. LV was hypertrophied with maximum wall thickness of 22±4 mm and nondilated (end-diastolic dimension, 39±7 mm). Cardiovascular magnetic resonance in 10 (of 15) patients showed no or minimal fibrosis (⩽5% LV mass). Elevated LV end-diastolic or pulmonary capillary wedge pressure, consistent with diastolic dysfunction, was present in 15 patients (75%). LV filling was impaired by echocardiographic measures in all patients, including a restrictive inflow pattern in 8 (40%). In 2 patients, traditional criteria for transplant were absent, including peak VO2 >14 mL/kg/min. Heart transplantation was performed in 12 patients with each alive and without cardiovascular symptoms, 2.3±1.7 years later. Conclusions—A previously under-recognized segment of the broad HCM clinical spectrum consists of nonobstructive patients with advanced heart failure, in the presence of preserved systolic function, for whom heart transplant is the sole definitive therapeutic option.Background— In hypertrophic cardiomyopathy (HCM), heart transplant has been predominantly confined to patients with systolic dysfunction. An underappreciated HCM subset comprises patients with preserved left ventricular (LV) systolic function who may also require consideration for transplantation. Therefore, we sought to define the clinical profile and occurrence of advanced heart failure among patients with nonobstructive HCM and preserved systolic function. Methods and Results— Databases from 2 referral centers comprising 2100 HCM patients were interrogated. Forty-six nonobstructive HCM patients (2.2%) either received or were listed for heart transplant, including 20 with normal systolic function (ejection fraction ≥50%). At transplant listing, these 20 patients were 42±13 years old, each in New York Heart Association functional class III/IV with ejection fraction of 62±7%. LV was hypertrophied with maximum wall thickness of 22±4 mm and nondilated (end-diastolic dimension, 39±7 mm). Cardiovascular magnetic resonance in 10 (of 15) patients showed no or minimal fibrosis (≤5% LV mass). Elevated LV end-diastolic or pulmonary capillary wedge pressure, consistent with diastolic dysfunction, was present in 15 patients (75%). LV filling was impaired by echocardiographic measures in all patients, including a restrictive inflow pattern in 8 (40%). In 2 patients, traditional criteria for transplant were absent, including peak VO2 >14 mL/kg/min. Heart transplantation was performed in 12 patients with each alive and without cardiovascular symptoms, 2.3±1.7 years later. Conclusions— A previously under-recognized segment of the broad HCM clinical spectrum consists of nonobstructive patients with advanced heart failure, in the presence of preserved systolic function, for whom heart transplant is the sole definitive therapeutic option.

Advanced Heart Failure With Preserved Systolic Function in Nonobstructive Hypertrophic CardiomyopathyCLINICAL PERSPECTIVE

Background—In hypertrophic cardiomyopathy (HCM), heart transplant has been predominantly confined to patients with systolic dysfunction. An underappreciated HCM subset comprises patients with preserved left ventricular (LV) systolic function who may also require consideration for transplantation. Therefore, we sought to define the clinical profile and occurrence of advanced heart failure among patients with nonobstructive HCM and preserved systolic function. Methods and Results—Databases from 2 referral centers comprising 2100 HCM patients were interrogated. Forty-six nonobstructive HCM patients (2.2%) either received or were listed for heart transplant, including 20 with normal systolic function (ejection fraction ≥50%). At transplant listing, these 20 patients were 42±13 years old, each in New York Heart Association functional class III/IV with ejection fraction of 62±7%. LV was hypertrophied with maximum wall thickness of 22±4 mm and nondilated (end-diastolic dimension, 39±7 mm). Cardiovascular magnetic resonance in 10 (of 15) patients showed no or minimal fibrosis (⩽5% LV mass). Elevated LV end-diastolic or pulmonary capillary wedge pressure, consistent with diastolic dysfunction, was present in 15 patients (75%). LV filling was impaired by echocardiographic measures in all patients, including a restrictive inflow pattern in 8 (40%). In 2 patients, traditional criteria for transplant were absent, including peak VO2 >14 mL/kg/min. Heart transplantation was performed in 12 patients with each alive and without cardiovascular symptoms, 2.3±1.7 years later. Conclusions—A previously under-recognized segment of the broad HCM clinical spectrum consists of nonobstructive patients with advanced heart failure, in the presence of preserved systolic function, for whom heart transplant is the sole definitive therapeutic option.Background— In hypertrophic cardiomyopathy (HCM), heart transplant has been predominantly confined to patients with systolic dysfunction. An underappreciated HCM subset comprises patients with preserved left ventricular (LV) systolic function who may also require consideration for transplantation. Therefore, we sought to define the clinical profile and occurrence of advanced heart failure among patients with nonobstructive HCM and preserved systolic function. Methods and Results— Databases from 2 referral centers comprising 2100 HCM patients were interrogated. Forty-six nonobstructive HCM patients (2.2%) either received or were listed for heart transplant, including 20 with normal systolic function (ejection fraction ≥50%). At transplant listing, these 20 patients were 42±13 years old, each in New York Heart Association functional class III/IV with ejection fraction of 62±7%. LV was hypertrophied with maximum wall thickness of 22±4 mm and nondilated (end-diastolic dimension, 39±7 mm). Cardiovascular magnetic resonance in 10 (of 15) patients showed no or minimal fibrosis (≤5% LV mass). Elevated LV end-diastolic or pulmonary capillary wedge pressure, consistent with diastolic dysfunction, was present in 15 patients (75%). LV filling was impaired by echocardiographic measures in all patients, including a restrictive inflow pattern in 8 (40%). In 2 patients, traditional criteria for transplant were absent, including peak VO2 >14 mL/kg/min. Heart transplantation was performed in 12 patients with each alive and without cardiovascular symptoms, 2.3±1.7 years later. Conclusions— A previously under-recognized segment of the broad HCM clinical spectrum consists of nonobstructive patients with advanced heart failure, in the presence of preserved systolic function, for whom heart transplant is the sole definitive therapeutic option.

37 Optimal Medical Management and Lowering LVAD Speed Prevents Progression of Aortic Insufficiency

Purpose: Development of aortic insufficiency (AI) in patients (pts) during continuous flow LVAD support is a well known phenomenon that can lead to ineffective LVAD output. Current literature reports an average AI progression by 0.7 grade at 6 months (mo). We hypothesized that optimal medical therapy (MT) together with lowering the LVAD speed to allow aortic valve (AV) opening delays development of significant AI. Methods and Materials: Charts and echocardiograms (echos) of 40 LVAD pts were reviewed for demographics and medications. Medications were uptitrated to MAP of 60-80mmHg. VAD speed (RPM) was reduced according to our outpatient VAD protocol and collected at 1 and 6 mo. Echos were reviewed at baseline (pre LVAD), 1 and 6 mo for left ventricular end diastolic dimensions (LVEDD)(cm), AV opening, presence/ absence of AI. AI was graded: O-none, 0.5-trace,1-mild,1.5-mild-moderate, 2-moderate, 2.5-moderate-severe, 3-severe. Results: Forty pts, mean age 58, were included in the study. At 6 mo 38/40 (93%) pts were receiving beta blocker (BB), 30/40 (73%) ACE-I/ARB, 27/40 (66%) aldosterone receptor blocker (ALDOBL). Fifty six percent of pts achieved half of maximal daily dose of ACE-I/ARB, 45% BB and 53% ALDOBL. Twenty nine out of 40 (71%) pts were on furosemide, mean dose 32 mg. At baseline mean LVEDD was 6.8, AI present in 12/40 pts (30%), mean grade 0.3. At 1 mo LVEDD decreased to a mean 5, AI present in 6/22 (27%) pts, mean grade 0.27. At 6 mo LVEDD was the same, mean 5.1, AI present in 8/19 (42%) pts. Mean grade increased from 0.27 to 0.4 (p 0.3). Mean LVAD speed at 1 mo was 9190 and decreased to 8840 at 6 mo (p 0.001). At 1 mo AV opened with every beat in 33% pts, partially opened in 8%, did not open in 54%. At 6 mo AV opened with every beat in 36% pts, partially opened in 21%, did not open in 43%. Conclusions: Our results suggest slower progression in the AI grade at 6 mo in LVAD pts, than reported to date. An early strategy of decreasing VAD speed combined with optimization of MT may account for this difference and possibly improve long term survival.