Successful Suture-Free Repair of Left Ventricular Rupture Using the EVARREST Patch.

Left ventricular free wall rupture is a lethal complication of myocardial infarction. Although emergent surgical repair is the treatment of choice, the method of repair remains highly individualized. This report presents a case of spontaneous coronary artery dissection in a patient with Turner syndrome that led to left ventricular free wall rupture and was successfully repaired on cardiopulmonary bypass using a suture-free technique with the EVARREST Fibrin Sealant Patch.

Outcomes of double-valve surgery for infective endocarditis are improving in the modern era.

BACKGROUND: The incidence of infective endocarditis (IE) is rapidly increasing. Contemporary outcomes following high-risk double valve surgery (DVS) for IE are not well described. METHODS: Between 2001 and 2021, 211 patients with IE underwent combined aortic and mitral valve surgery at a tertiary care referral center. Data from the Society of Thoracic Surgeons registry, including demographics, operative details, and outcomes, were collected. Risk factors for 30-day and 1-year-mortality were analyzed. Survival was analyzed using Kaplan-Meier and Cox proportional hazards modeling. RESULTS: The study cohort had a male preponderance (73%), with a median age of 56 years (interquartile range [IQR], 44 to 63 years). Forty-five patients (21%) had a history of intravenous (IV) drug abuse, 50 (24%) were on preoperative dialysis, and 50 (24%) had prosthetic valve endocarditis. Thirty-day and 1-year mortality were 14% (n = 30) and 30% (n = 61), respectively. On multivariable Cox regression adjusting for age, prosthetic valve endocarditis, postoperative intra-aortic balloon pump (IABP), history of dialysis (adjusted hazard ratio [aHR], 1.9; 95% confidence interval [CI], 1.3 to 2.9; P = .002) and IV drug abuse (aHR, 2.0; 95% CI, 1.1-3.5; P = .02) were predictive of decreased survival. Undergoing surgery after 2010 was predictive of improved survival (aHR, 0.5; 95% CI, 0.3 to 0.8; P = .006). These patients were more likely to undergo urgent/emergent surgery (83% vs 29%; P < .001) and less likely to have an aortic root abscess (40% vs 58%; P = .03) or to require the commando procedure (13% vs 33%; P = .002). CONCLUSIONS: In this large series evaluating outcomes of DVS for IE in the modern era, although the mortality risk remained elevated, improving outcomes may be associated with earlier surgical intervention before significant disease progression. Multidisciplinary evaluation for complex IE may be considered to better understand the optimal timing and repair strategy.

Evolution of Pulmonary Valve Management During Repair of Tetralogy of Fallot: A 14-year Experience.

BACKGROUND: The optimal repair strategy for tetralogy of Fallot remains controversial. This report presents a 14-year evolution of management of the pulmonary valve (PV) from transannular patch to valve-sparing repair to neovalve creation using living right atrial appendage tissue. METHODS: A retrospective review of 172 consecutive patients undergoing complete repair for TOF between January 2007 and June 2021 was performed. Clinical and follow-up data were analyzed by repair group. Neopulmonary valve (NPV) creation using right atrial appendage tissue was introduced in 2019. Failure of valve-sparing repair was defined as needing reintervention for recurrent right ventricular outflow tract obstruction (RVOTO). RESULTS: Median age and weight at repair were 4.9 months and 6 kg, respectively. Median preoperative PV size and z-score were 6.4 mm (5.2-8.3 mm) and -3.2 (-4.1 to -2.1), respectively. Patients who underwent valve-sparing repair had larger PV size and z-score compared with patients who underwent transannular patch procedures (8 mm vs 5.6 mm; -2.1 vs -3.2; both P < .001). There were no hospital mortalities. Overall follow-up was 44 months. At last follow-up, 10% of patients who underwent valve-sparing repair had repeat intervention for recurrent RVOTO. Patients who had failed valve-sparing repair had significantly lower PV z-scores (-2.6 vs -1.9; P = .01). An NPV was used in 8 patients with a median PV z-score of -4 (-4.7 to -3.9). At 6 months, 6 patients (75%) had mild or trivial pulmonary insufficiency after NPV placement. CONCLUSIONS: Repair of tetralogy of Fallot is a safe operation with excellent outcomes. Valve-sparing repair avoids right ventricular dilation but may fail for RVOTO at a PV z-score <-2. NPV creation offers an alternative option in patients with a small PV.

Technique for Neo-Pulmonary Valve Creation With Living Tissue for Repair of Atrioventricular Septal Defect and Tetralogy of Fallot.

Long-standing effects of pulmonary regurgitation after transannular patch repair in Tetralogy of Fallot (ToF) can be especially deleterious in the setting of combined ToF and complete atrioventricular septal defect (CAVSD). We present a technique for a complete repair of combined ToF/CAVSD using right atrial appendage tissue to create a competent neo-pulmonary valve. This technique provides advantages of right heart protection via pulmonary valve competence and the use of living tissue capable of growth with the patient, potentially obviating the need for repeat interventions.

LPGAT1 controls the stearate/palmitate ratio of phosphatidylethanolamine and phosphatidylcholine in sn-1 specific remodeling.

Most mammalian phospholipids contain a saturated fatty acid at the sn-1 carbon atom and an unsaturated fatty acid at the sn-2 carbon atom of the glycerol backbone group. While the sn-2 linked chains undergo extensive remodeling by deacylation and reacylation (Lands cycle), it is not known how the composition of saturated fatty acids is controlled at the sn-1 position. Here, we demonstrate that lysophosphatidylglycerol acyltransferase 1 (LPGAT1) is an sn-1 specific acyltransferase that controls the stearate/palmitate ratio of phosphatidylethanolamine (PE) and phosphatidylcholine. Bacterially expressed murine LPGAT1 transferred saturated acyl-CoAs specifically into the sn-1 position of lysophosphatidylethanolamine (LPE) rather than lysophosphatidylglycerol and preferred stearoyl-CoA over palmitoyl-CoA as the substrate. In addition, genetic ablation of LPGAT1 in mice abolished 1-LPE:stearoyl-CoA acyltransferase activity and caused a shift from stearate to palmitate species in PE, dimethyl-PE, and phosphatidylcholine. Lysophosphatidylglycerol acyltransferase 1 KO mice were leaner and had a shorter life span than their littermate controls. Finally, we show that total lipid synthesis was reduced in isolated hepatocytes of LPGAT1 knockout mice. Thus, we conclude that LPGAT1 is an sn-1 specific LPE acyltransferase that controls the stearate/palmitate homeostasis of PE and the metabolites of the PE methylation pathway and that LPGAT1 plays a central role in the regulation of lipid biosynthesis with implications for body fat content and longevity.

A Bayesian Analysis to Determine the Prevalence of Barth Syndrome in the Pediatric Population.

OBJECTIVE: To determine the prevalence of Barth syndrome in the pediatric population. STUDY DESIGN: Data were collected from the Barth Syndrome Foundation Registry and relevant literature. With the advent of genetic testing and whole-exome sequencing, a multipronged Bayesian analysis was used to estimate the prevalence of Barth syndrome based on published data on the incidence and prevalence of cardiomyopathy and neutropenia, and the respective subpopulations of patients with Barth syndrome indicated in these publications. RESULTS: Based on 7 published studies of cardiomyopathy and 2 published studies of neutropenia, the estimated prevalence of Barth syndrome is approximately 1 case per million male population. This contrasts with 99 cases in the Barth Syndrome Foundation Registry, 58 of which indicate a US location, and only 230-250 cases known worldwide. CONCLUSIONS: It appears that Barth syndrome is greatly underdiagnosed. There is a need for better education and awareness of this rare disease to move toward early diagnosis and treatment.

A critical appraisal of the tafazzin knockdown mouse model of Barth syndrome: what have we learned about pathogenesis and potential treatments?

Pediatric heart failure remains poorly understood, distinct in many aspects from adult heart failure. Limited data point to roles of altered mitochondrial functioning and, in particular, changes in mitochondrial lipids, especially cardiolipin. Barth syndrome is a mitochondrial disorder caused by tafazzin mutations that lead to abnormal cardiolipin profiles. Patients are afflicted by cardiomyopathy, skeletal myopathy, neutropenia, and growth delay. A mouse model of Barth syndrome was developed a decade ago, which relies on a doxycycline-inducible short hairpin RNA to knock down expression of tafazzin mRNA (TAZKD). Our objective was to review published data from the TAZKD mouse to determine its contributions to our pathogenetic understanding of, and potential treatment strategies for, Barth syndrome. In regard to the clinical syndrome, the reported physiological, biochemical, and ultrastructural abnormalities of the mouse model mirror those in Barth patients. Using this model, the peroxisome proliferator-activated receptor pan-agonist bezafibrate has been suggested as potential therapy because it ameliorated the cardiomyopathy in TAZKD mice, while increasing mitochondrial biogenesis. A clinical trial is now underway to test bezafibrate in Barth syndrome patients. Thus the TAZKD mouse model of Barth syndrome has led to important insights into disease pathogenesis and therapeutic targets, which can potentially translate to pediatric heart failure.