Influence of donor age and donor-recipient age difference on intimal hyperplasia in pediatric patients with young and adult donors vs. adult patients after heart transplantation.

AIM: Optimal selection and allocation of donor hearts is a relevant aspect in transplantation medicine. Donor age and cardiac allograft vasculopathy (CAV) affect post-transplant mortality. To what extent donor age impacts intimal hyperplasia (CAVIH) in pediatric and adult patients after heart transplantation (HTx) is understudied. METHODS: In a cohort of 98 HTx patients, 58 pediatric (24.1% with adult donors) and 40 adult patients, we assessed the effect of donor age and donor-recipient age difference (D-R) on the continuous parameter of maximal intima thickness (mIT) in optical coherence tomography. We evaluated their predictive value regarding higher mIT and the prevalence of CAVIH, defined as mIT > 0.3 mm, and compared it to established CAV risk factors. RESULTS: In the overall population, donor age correlated with mIT (p < 0.001), while in the pediatric subpopulation, both donor age and D-R correlated with mIT (p < 0.001 and p = 0.002, respectively). In the overall population, donor age was a main predictor of higher mIT and CAVIH (p = 0.001 and p = 0.01, respectively) in addition to post-transplant interval, arterial hypertension, and dyslipidemia. In the pediatric patients, dyslipidemia remained a main predictor of both higher mIT and CAVIH (p = 0.004 and p = 0.040, respectively), while donor age and D-R were not. CONCLUSION: While there was an effect of the non-modifiable parameter of donor age regarding maximal intimal thickness, a stronger association was seen between the modifiable risk factor dyslipidemia and higher maximal intimal thickness and CAVIH in both the overall population and the pediatric subpopulation.

Gigantic biatrial myxoma in a young female with an atrial septal defect: a case report.

Background: Myxomas are uncommon and benign cardiac neoplasms that can present with various cardiac, systemic, embolic, or without symptoms depending on their location and size. Very few cases of large, truly biatrial, or tumours connected via the cardiac atria have been reported throughout the years. Case summary: We present an unusual case of an apparently healthy 25-year-old French woman, who presented with dyspnoea at Munich's Octoberfest. Echocardiography and computed tomography identified gigantic masses in left and right atrium, which were connected through an atrial septal defect. They were successfully removed by emergent cardiac surgery. Discussion: This case describes an uncommon tumour and highlights the importance of a quick diagnosis and prompt surgery. We describe the management and surgery for atrial myxomas as well as demonstrating pre- and intraoperative pictures.

Mid-Term Outcome after Extracorporeal Life Support in Postcardiotomy Cardiogenic Shock: Recovery and Quality of Life.

Background: Extracorporeal life support (ECLS) therapy for refractory postcardiotomy cardiogenic shock (rPCS) is associated with high early mortality rates. This study aimed to identify negative predictors of mid-term survival and to assess health-related quality of life (HRQoL) and recovery of the survivors. Methods: Between 2017 and 2020, 142 consecutive patients received ECLS therapy following cardiac surgery. The median age was 66.0 [57.0-73.0] years, 67.6% were male and the median EuroSCORE II was 10.5% [4.2-21.3]. In 48 patients, HRQoL was examined using the 36-Item Short Form Survey (SF-36) and the modified Rankin-Scale (mRS) at a median follow-up time of 2.2 [1.9-3.2] years. Results: Estimated survival rates at 3, 12, 24 and 36 months were 47%, 46%, 43% and 43% (SE: 4%). Multivariable Cox Proportional Hazard regression analysis revealed preoperative EuroSCORE II (p = 0.013), impaired renal function (p = 0.010), cardiopulmonary bypass duration (p = 0.015) and pre-ECLS lactate levels (p = 0.004) as independent predictors of mid-term mortality. At the time of follow-up, 83.3% of the survivors were free of moderate to severe disability (mRS < 3). SF-36 analysis showed a physical component summary of 45.5 ± 10.2 and a mental component summary of 50.6 ± 12.5. Conclusions: Considering the disease to be treated, ECLS for rPCS is associated with acceptable mid-term survival, health-related quality of life and functional status. Preoperative EuroSCORE II, impaired renal function, cardiopulmonary bypass duration and lactate levels prior to ECLS implantation were identified as negative predictors and should be included in the decision-making process.

Do it "RIGHT": HeartMate 3 as Destination Therapy Right Ventricular Assist Device in a Patient With Arrhythmogenic Right Ventricular Cardiomyopathy.

Isolated right ventricular failure presents a rare pathology and heart transplantation remains the treatment of choice. Because of the shortage of donor organs, alternative treatment options are required. However, current continuous-flow ventricular assist devices are designed and approved only for left ventricular support and right ventricular implantation usually presents a procedure of last resort. Herein, we present a successful implantation of the HeartMate 3 for isolated right ventricular support as destination therapy in a patient suffering from late-onset arrhythmogenic right ventricular cardiomyopathy.

Myocarditis or inherited disease? - The multifaceted presentation of arrhythmogenic cardiomyopathy.

INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is now usually referred to as arrhythmogenic cardiomyopathy (ACM) because of the possible left and biventricular affection. In recent years, it has been shown that early-stage ACM, especially in women carrying a disease-causing variant in the DSP gene, may present with clinical signs of myocarditis. CASE PRESENTATION: The female patient was diagnosed with myocarditis based on arrhythmia and findings on magnetic resonance imaging at the age of 24 years. An additional performed myocardial biopsy confirmed a lymphocytic inflammatory reaction. Subsequently, the patient experienced cardiac arrest because of ventricular fibrillation and was resuscitated. As a result, she received an implantable cardioverter defibrillator, and repeated ablations of recurrent ventricular tachycardia were performed. After four years, molecular genetic testing identified the heterozygous, likely pathogenic nonsense variant c.4789G > T, p.(Glu1597*) in DSP (NM_004415.4). Based on this finding, ACM could be diagnosed, and a heart transplantation was performed only a few months later because of rapid disease progression. DISCUSSION: Truncating variants in DSP have been associated with fulminant progression of arrhythmia. However, the currently used ARVC task force criteria are inadequate to detect DSP-associated ACM with left dominant presentation. Moreover, the initial diagnosis of myocarditis may distract from a more extensive search for other causes. Consequently, in cases of recurrent or unusually prolonged myocarditis, especially if present without detected pathogens, molecular genetic testing should be considered.

German Aortic Root Repair Registry-Insights From the First 400 Consecutive Patients.

BACKGROUND: The objective was to provide initial data from our prospective valve-sparing aortic root replacement (V-SARR) registry and reasons for conversion to prosthetic aortic valve replacement. METHODS: Six centers established an intention-to-treat-design V-SARR-registry (the German Aortic Root Repair Registry; first patient in October 2016); the main inclusion criterion was being scheduled for V-SARR as plan A. Clinical information, operative details, intraoperative valve/root measurements, and clinical and transthoracic echocardiography follow-up-data are documented. RESULTS: Of a total of 449 patients, we report data for 401 (81% male; mean age 51 ± 14 years). Overall, 350 patients underwent V-SARR as scheduled, group A (David variants I 55%, III 2%, IV 13%, V 24%, V-Stanford 2%, and Yacoub remodeling 2%); and 51 were converted to aortic valve replacement (group B). Median follow-up was 11 months (range, 0 to 2.6 years), cumulative follow-up was 279 patient-years. In group B, there were fewer connective tissue disorders (6% vs 16%), fewer patients had left ventricular ejection fraction greater than 50% (60% vs 90%), more had bicuspid aortic valves (45% vs 28%), and fewer patients had preoperative none/trace aortic regurgitation (2% vs 20%). Fewer patients in group B had rare types of bicuspid aortic valve (fused N/L, R/N, 10% vs 30%) and more had unbalanced roots (56% vs 40%). Immediate postoperative aortic regurgitation was none/trace in 79% and mild in 20%. At 30 days, the mean transvalvular pressure gradient was 7 ± 5 mm Hg. None of the patients died in hospital; two strokes occurred. One patient needed early aortic valve replacement as redo surgery. CONCLUSIONS: The main factors causing surgeons to convert a planned V-SARR to aortic valve replacement include asymmetry of aortic valve/root, severity of aortic regurgitation, safety reasons (left ventricular ejection fraction), and bicuspid aortic valves (but not rare types). The German Aortic Root Repair Registry will help us identify the impact on long-term outcomes of preoperative and postoperative valvular anatomy and various V-SARR types.

Case report: Heart Mate III for systemic right ventricular support in a patient with hypoplastic left heart syndrome.

Ventricular assist device implantation presents a possible bridge to heart transplantation for patients with failing Fontan physiology. However, evidence regarding outcome and possible pitfalls associated with the Fontan circulation is still insufficient. We describe the course of a 13-year-old male, who was born with hypoplastic left heart syndrome and underwent HeartMate III implantation due to refractory failure of the systemic right ventricle.

The collateral network concept: a reassessment of the anatomy of spinal cord perfusion.

OBJECTIVE: Prevention of paraplegia after repair of thoracoabdominal aortic aneurysm requires understanding the anatomy and physiology of the spinal cord blood supply. Recent laboratory studies and clinical observations suggest that a robust collateral network must exist to explain preservation of spinal cord perfusion when segmental vessels are interrupted. An anatomic study was undertaken. METHODS: Twelve juvenile Yorkshire pigs underwent aortic cannulation and infusion of a low-viscosity acrylic resin at physiologic pressures. After curing of the resin and digestion of all organic tissue, the anatomy of the blood supply to the spinal cord was studied grossly and with light and electron microscopy. RESULTS: All vascular structures at least 8 µm in diameter were preserved. Thoracic and lumbar segmental arteries give rise not only to the anterior spinal artery but to an extensive paraspinous network feeding the erector spinae, iliopsoas, and associated muscles. The anterior spinal artery, mean diameter 134 ± 20 µm, is connected at multiple points to repetitive circular epidural arteries with mean diameters of 150 ± 26 µm. The capacity of the paraspinous muscular network is 25-fold the capacity of the circular epidural arterial network and anterior spinal artery combined. Extensive arterial collateralization is apparent between the intraspinal and paraspinous networks, and within each network. Only 75% of all segmental arteries provide direct anterior spinal artery-supplying branches. CONCLUSIONS: The anterior spinal artery is only one component of an extensive paraspinous and intraspinal collateral vascular network. This network provides an anatomic explanation of the physiological resiliency of spinal cord perfusion when segmental arteries are sacrificed during thoracoabdominal aortic aneurysm repair.

The collateral network concept: remodeling of the arterial collateral network after experimental segmental artery sacrifice.

OBJECTIVE: A comprehensive strategy to prevent paraplegia after open surgical or endovascular repair of thoracoabdominal aortic aneurysms requires a thorough understanding of the response of the collateral network to extensive segmental artery sacrifice. METHODS: Ten Yorkshire pigs underwent perfusion with a low-viscosity acrylic resin. With the use of cardiopulmonary bypass, 2 animals each were perfused in the native state and immediately, 6 hours, 24 hours, and 5 days after sacrifice of all segmental arteries (T4-L5). After digestion of surrounding tissue, the vascular cast of the collateral network underwent analysis of arterial and arteriolar diameters and the density and spatial orientation of the vasculature using light and scanning electron microscopy. RESULTS: Within 24 hours, the diameter of the anterior spinal artery had increased significantly, and within 5 days the anterior spinal artery and the epidural arterial network had enlarged in diameter by 80% to 100% (P < .0001). By 5 days, the density of the intramuscular paraspinous vessels had increased (P < .0001), a shift of size distribution from small to larger arterioles was seen (P = .0002), and a significant realignment of arterioles parallel to the spinal cord had occurred (P = .0005). CONCLUSIONS: Within 5 days after segmental artery occlusion, profound anatomic alterations in the intraspinal and paraspinous arteries and arterioles occurred, providing the anatomic substrate for preservation of spinal cord blood flow via collateral pathways.

Staged repair significantly reduces paraplegia rate after extensive thoracoabdominal aortic aneurysm repair.

OBJECTIVE: Paraplegia remains a devastating, and still too frequent, complication after repair of extensive thoracoabdominal aortic aneurysms. Strategies to prevent ischemic spinal cord damage after extensive segmental artery sacrifice-or occlusion, essential for endovascular repair-are still evolving. METHODS: Ninety patients who underwent extensive segmental artery sacrifice (median, 13; range, 9-15) during open surgical repair from June 1994 to December 2007 were reviewed retrospectively. Fifty-five patients (mean age, 65 +/- 12 years; 49% were male), most with extensive Crawford type II thoracoabdominal aortic aneurysms, had a single procedure (single-stage group). Thirty-five patients (mean age, 62 +/- 14 years; 57% were male) had 2 procedures (2-stage group), usually Crawford type III or IV repair after operation for Crawford type I descending thoracic aneurysm. The median interval between the 2-stage procedures was 5 years (3 months to 17 years). There were no significant differences between the groups with regard to age, gender, cause of the aneurysm, hypertension, chronic obstructive pulmonary disease, urgency, previous cerebrovascular accidents, year of procedure, or cerebrospinal fluid drainage. In single-stage procedures, hypothermic circulatory arrest was used in 29% of patients, left-sided heart bypass was used in 40% of patients, and partial cardiopulmonary bypass was used in 27% of patients. Somatosensory-evoked potentials were monitored in all patients, and motor-evoked potentials were monitored in 39% of patients. Cerebrospinal fluid was drained in 84% of patients. RESULTS: Overall hospital mortality was 11.1%. There were no significant differences in mortality, stroke, postoperative bleeding, infection, renal failure, or pulmonary insufficiency between the groups. However, 15% of patients in the single-stage group had permanent spinal cord injury versus none in the 2-stage group (P = .02). The significantly lower rate of paraplegia and paraparesis in the 2-stage group occurred despite a significantly higher number of segmental arteries sacrificed in this group: a median of 14 (11-15) versus 12 (9-15) (P < .0001). CONCLUSION: A staged approach to extensive thoracoabdominal aortic aneurysm repair may reduce the incidence of spinal cord injury. This is of particular importance in designing strategies involving hybrid or entirely endovascular procedures.

Long-term survival after open repair of chronic distal aortic dissection.

BACKGROUND: The optimal treatment of chronic distal aortic dissection remains controversial, with endovascular stent-graft techniques challenging traditional surgery. METHODS: From January 1994 to April 2007, 104 patients (82 male, median age 60.5 years) with chronic distal aortic dissection underwent surgical repair, 0 to 21 years after initial diagnosis of acute type A or B dissection (median 2.1 years). Twenty-three (22%) patients underwent urgent-emergent surgery. Mean aortic diameter was 6.9 +/- 1.4 cm. Indications for surgery, other than aortic expansion, were pain in 6 (6%) patients, malperfusion in 6 (6%), and rupture in 11 (11%). Forty-nine (47%) had previous cardioaortic surgery (29% dissection-related), 21 (20%) had coronary artery disease, 12 (12%) had Marfan syndrome, and 4 (4%) were on chronic dialysis. Twenty-six (25%) had a thrombosed false lumen. Thirty (29%) patients required reimplantation of visceral arteries; 8.3 +/- 2.7 segmental artery pairs were sacrificed. RESULTS: Hospital mortality was 9.6% (10 patients). Paraplegia occurred in 5 (4.8%). Twenty-seven patients (26%) experienced adverse outcome (death within one year, paraplegia, stroke, or dialysis). Adverse outcome was associated with atheroma (p = 0.04, odds ratio = 4.3). Survival was 78% at 1, 68% at 5, and 59% at 10 years (average follow-up, 7.7 +/- 4.1 years). Freedom from distal aortic reoperation was 99% at 1, 93% at 5, and 83% at 10 years. After one year, patients enjoyed longevity equivalent to a normal age-sex matched population (standardized mortality ratio = 1.38, p = 0.23). By multivariate analysis, atheroma (p = 0.0005, relative risk = 9.32) and age (p = 0.0003, relative risk = 1.15/year) were risk factors for long-term survival. CONCLUSIONS: The efficacy of open repair for distal chronic dissection is highlighted by normal survival after the first year, and a low reoperation-reintervention rate.

Redo lateral thoracotomy for reoperative descending and thoracoabdominal aortic repair: a consecutive series of 60 patients.

BACKGROUND: Reoperative descending thoracic aorta (DTA) or thoracoabdominal aortic aneurysm (TAAA) surgery is a challenge because of increased risk of lung injury and diffuse bleeding. METHODS: Sixty patients (34 male, mean age 54.4 years) underwent redo thoracotomy for DTA (22 patients) or extended thoracoabdominal incision for reoperative TAAA (38 patients) from March 1988 to June 2007, after 1.7 +/- 0.9 previous cardioaortic procedures. Forty-one patients were hypertensive (68%), 18 were smokers (30%), 9 had Marfan syndrome (15%), 9 had coronary artery disease (15%), 5 had chronic obstructive pulmonary disease (8%), and 3 had diabetes mellitus (5%). In all, 45% (27 patients) had previous dissection, 30% (18) had atherosclerotic aneurysms, 15% had coarctation surgery (9), and 6 patients had other etiologies. Mean follow-up, 100% complete, was 6.5 years. RESULTS: Hospital mortality for reoperative DTA/TAAA was 13.3% (8 patients). Although 6.3 +/- 2.9 (0 to 14) segmental artery pairs were sacrificed at reoperation-and 6.2 +/- 2.3 (1 to 12) initially-for a total of 10.6 +/- 3.9 (2 to 15) segmental artery pairs sacrificed, only 1 patient had paraplegia (1.6%). Four patients had a 2-day procedure, with 12 to 24 hours of intensive care unit recovery after lysis of extensive adhesions: all survived. Respiratory complications occurred in 13 patients (21.6%), and permanent dialysis was required in 2 (3.3%), but there were no strokes. Adverse outcome-1-year mortality, stroke, permanent dialysis, or paraplegia-occurred in 13 patients (21.6%). Adverse outcome was marginally associated (p < 0.2) with increased age, atherosclerotic aneurysms (33% versus 17% other), TAA incision (30% versus 9%), and greater aneurysm extent, and was significantly associated with perfusion technique (p = 0.02). Adverse outcome occurred in 3 of 4 patients who had clamp-and-sew technique, 6 of 21 using partial cardiopulmonary bypass (28.6%), and 3 of 17 with partial left heart bypass (17.7%), but only 1 of 18 with hypothermic circulatory arrest (5.6%). CONCLUSIONS: Reoperative DTA/TAAA repair was significantly safer with hypothermic circulatory arrest rather than partial cardiopulmonary bypass, partial left heart bypass, or clamp-and-sew strategy. A 2-day procedure may be advisable for patients with extensive adhesions.