The so-called "one-and-a-half" ventricular repair: where are we after 40 years?

OBJECTIVES: The indications, timing, and results of the so-called "one-and-a-half ventricle repair", as a surgical alternative to the creation of the Fontan circulation, or high-risk biventricular repair, currently remain nebulous. We aimed to clarify these issues. METHODS: We reviewed a total of 201 investigations, assessing selection of candidates, the need for atrial septal fenestration, the fate of an unligated azygos vein and free pulmonary regurgitation, the concerns regarding reverse pulsatile flow in the superior caval vein, the growth potential and function of the subpulmonary ventricle, and the role of the superior cavopulmonary connections as an interstage procedure prior to biventricular repair, or as a salvage procedure. We also assessed subsequent eligibility for conversion to biventricular repair and long-term functional results. RESULTS: Reported operative mortalities ranged from 3% to 20%, depending on the era of surgical repair with 7% risk of complications due to a pulsatile superior caval vein, up to one-third incidence of supraventricular arrhythmias, and a small risk of anastomotic takedown of the superior cavopulmonary connection. Actuarial survival was between 80% and 90% at 10 years, with two-thirds of patients in good shape after 20 years. We found no reported instances of plastic bronchitis, protein-losing enteropathy, or hepatic cirrhosis. CONCLUSIONS: The so-called "one-and-a-half ventricular repair", which is better described as production of one-and-a-half circulations can be performed as a definitive palliative procedure with an acceptable risk similar to that of conversion to the Fontan circulation. The operation reduces the surgical risk for biventricular repair and reverses the Fontan paradox.

Effect of Yoga on Clinical Outcomes and Quality of Life in Patients With Vasovagal Syncope (LIVE-Yoga).

OBJECTIVES: This study aims to determine the impact of yoga as an adjunct to standard therapy versus standard therapy alone on the symptomatic burden in patients with recurrent vasovagal syncope (VVS). BACKGROUND: There is a significant reduction in the quality of life (QoL) of patients with recurrent VVS. Existing management therapies have been largely ineffective. Recent trials have demonstrated the efficacy of yoga in diseases with autonomic imbalance, suggesting its possible utility in VVS. METHODS: Patients with recurrent VVS were randomized to receive either a specialized yoga training program in addition to current guideline-based therapy (intervention arm, group 1) or current guideline-based therapy alone (control arm, group 2). The primary outcome was a composite of the number of episodes of syncope and presyncope at 12 months. Secondary outcomes included QoL assessment by World Health Organization Quality of Life Brief Field questionnaire (WHOQoL-BREF) scores and Syncope Functional Status Questionnaire scores at 12 months, head up tilt test, and heart rate variability at 6 weeks. RESULTS: A total of 55 patients underwent randomization. The mean number of syncopal or presyncopal events at 12 months was 0.7 ± 0.7 in the intervention arm compared to 2.52 ± 1.93 in the control arm (P < 0.01). In the intervention arm, 13 (43.3%) patients remained free of events versus 4 (16.0%) patients in the control arm (P = 0.02). QoL at 12 months showed significant improvement of all Syncope Functional Status Questionnaire scores and 2 domains of WHOQoL-BREF scores (P < 0.05). CONCLUSIONS: Yoga as adjunctive therapy is superior to standard therapy alone in reducing the symptomatic burden and improving QoL in patients with recurrent VVS.

Concomitant Transthyretin Amyloidosis and Severe Aortic Stenosis in Elderly Indian Population: A Pilot Study.

BACKGROUND: Prevalence of both degenerative severe aortic stenosis (AS) and transthyretin cardiac amyloidosis (ATTR-CA) increases with age. Dual disease (AS+myocardial ATTR-CA) occurs in significant proportion of patients undergoing surgical aortic valve replacement (SAVR). OBJECTIVES: This study aimed to determine the prevalence of ATTR-CA in severe AS in the Indian population, identify noninvasive predictors of its diagnosis, and understand its impact on prognosis. METHODS: Symptomatic severe AS patients aged ≥65 years undergoing SAVR were enrolled. ATTR-CA diagnosis was based on preoperative 99m-technetium pyrophosphate (PYP) scan and intraoperatively obtained basal interventricular septum biopsy for myocardial ATTR-CA, and excised native aortic valve for isolated valvular ATTR-CA. Primary amyloidosis was excluded by serum/urine protein electrophoresis with serum immunofixation. RESULTS: SAVR was performed in 46 AS patients (age 70 ± 5 years, 70% men). PYP scan was performed for 32 patients, with significant PYP uptake in 3 (n = 3 of 32, 9.4%), suggestive of myocardial ATTR-CA. On histopathological examination, none of the interventricular septum biopsy specimens had amyloid deposits, whereas 33 (71.7%) native aortic valves showed amyloid deposits, of which 19 (57.6%) had transthyretin deposition suggestive of isolated valvular amyloidosis. Noninvasive markers of dual disease included low myocardial contraction fraction (median [interquartile range], 28.8% [23.8% to 39.1%] vs 15.3% [9.3% to 16.1%]; P = 0.006), deceleration time (215 [144 to 236] ms vs 88 [60 to 106] ms; P = 0.009) and global longitudinal strain (-18.7% [-21.1% to -16.9%] vs -14.2% [-17.0% to -9.7%]; P = 0.030). At 1-year follow-up, 2 patients died (4.3%); 1 each in myocardial ATTR-CA negative and positive groups (3.4% vs 33.3%; P = 0.477). CONCLUSIONS: Dual disease is not uncommon in India. Isolated valvular amyloidosis in severe AS is much more common.

Cardiovigilance.

Cardiovascular disease (CVD) is a major cause of morbidity and mortality across the globe. Prevention and management of the CVD pandemic calls for concerted action on part of all health care professionals, as well as other concerned stakeholders. We call for cardiovigilance in healthcare and define it as "the action or state of keeping careful watch, to prevent, screen, diagnose and manage cardiovascular disease (CVD) in a timely and appropriate manner". We expand upon the concept of cardiovigilance, describe its utility, and suggest various taxonomic rubrics to simplify its practice.

Proposed low-density lipoprotein cholesterol goals for secondary prevention and familial hypercholesterolemia in India with focus on PCSK9 inhibitor monoclonal antibodies: Expert consensus statement from Lipid Association of India.

BACKGROUND: Rates of atherosclerotic cardiovascular disease (ASCVD) are strikingly high in India compared to Western countries and are increasing. Moreover, ASCVD events occur at a younger age with only modest hypercholesterolemia, most commonly with low levels of high-density lipoprotein cholesterol. The course of ASCVD also appears to be more fulminant with higher mortality. OBJECTIVE: In light of these issues, the Lipid Association of India (LAI) endeavored to develop revised guidelines with more aggressive low-density lipoprotein cholesterol (LDL-C) goals in secondary prevention and for patients with familial hypercholesterolemia compared to guidelines in the United States and other countries. METHODS: Owing to the paucity of clinical outcomes data in India, it was necessary to place major emphasis on expert opinion as a complement to randomized placebo-controlled data generated mostly in non-Indian cohorts. To facilitate this process, the LAI conducted a series of 19 meetings among 162 lipid specialists in 13 cities throughout India over a period of 11 months before formulating this expert consensus statement. RESULTS: The LAI recommends an LDL-C goal <50 mg/dL in all patients in secondary prevention or very high-risk primary prevention but proposes an optional goal ≤30 mg/dL in category A extreme-risk patients (eg, coronary artery disease + familial hypercholesterolemia) and a recommended goal ≤30 mg/dL in category B extreme-risk patients [coronary artery disease + (1) diabetes and polyvascular disease/≥3 major ASCVD risk factors/end organ damage, or (2) recurrent acute coronary syndrome within 12 months despite LDL-C <50 mg/dL, or (3) homozygous familial hypercholesterolemia]. CONCLUSIONS: More aggressive LDL-C goals are needed for prevention of ASCVD in India, as described in this expert consensus statement. Use of statins and ezetimibe needs to increase in India in combination with improved control of other ASCVD risk factors. Proprotein convertase subtilisin kexin type 9 inhibitors can improve LDL-C goal achievement in patients with refractory hypercholesterolemia.