Inverted left atrial appendage mimicking potential left atrial thrombus in an infant following cardiac surgery: a case report.

Background: Inverted left atrial appendage (iLAA) is an infrequent complication following cardiac surgery, seen both in children and adults. Following a recent encounter, this review article is aimed to remind the reader about its occurrence, clinical manifestations, differential diagnoses, and management options. Case summary: A 3-month-old baby underwent successful surgical repair of a large ventricular septal defect. Intraoperative epicardial echocardiogram at the end of the case demonstrated an unexpected left atrial mass, raising suspicion of a thrombus adjacent to the mitral valve. Urgent re-establishment of cardiopulmonary bypass and exploration of the left atrium did not reveal a thrombus, but this was subsequently identified as an iLAA. This completely resolved after manual external reduction of the appendage. Discussion: Published literature is confined to case reports only, with most cases observed post-operatively, but some occurring spontaneously. Awareness of this unusual manifestation is particularly important in the intraoperative period as it can usually be addressed without the need for further cardiopulmonary bypass.

Outcomes of surgery for extensive infective endocarditis.

OBJECTIVE: Extensive infective endocarditis (EIE) involving the valve annulus or the intervalvular fibrous body (IFB) is a treatment challenge. We sought to clarify the outcomes of patients undergoing surgery for EIE. METHODS: We retrospectively reviewed all 197 consecutive patients who underwent an operation for infective endocarditis (IE) between 2005 and 2016 in the Helsinki University Hospital. Thirty-five (18%) patients had EIE, of which 17 (9%) infection extended to IFB. RESULTS: Patients with EIE had higher EuroSCORE II (24.4% vs. 12.4% p < .001), higher frequency of diabetes (29% vs. 13% p = .017), more often NYHA Class IV (83% vs. 56% p = .02), aortic (97% vs. 45% p < .001), multivalve (40% vs. 11% p < .001), and prosthetic valve IE (37% vs. 9% p < .001), and underwent more often emergency surgery (46% vs. 29% p = .042). Thirty-day mortality was 9% in the EIE group and 7% in the non-EIE group (p = .720). Survival of patients with EIE at 5 years was 60% and with non-EIE 71% (p = .029). The frequency of complications was higher in EIE (54%) than in non-EIE patients (25%) (p < .001), due to the higher need for permanent pacemaker implantations (34% vs. 4% p < .001). Freedom from re-operations at 5 years was 91% in the EIE group and 97% in the non-EIE group (p = .203). CONCLUSIONS: Early mortality of surgery for EIE was comparable with non-EIE. Midterm survival was lower after surgery for EIE than after surgery for non-EIE but there was no difference in survival of patients with IE limited to the valve annulus amenable to patch repair and patients with endocarditis requiring IFB reconstruction.

Epitranscriptomics of Ischemic Heart Disease-The IHD-EPITRAN Study Design and Objectives.

Epitranscriptomic modifications in RNA can dramatically alter the way our genetic code is deciphered. Cells utilize these modifications not only to maintain physiological processes, but also to respond to extracellular cues and various stressors. Most often, adenosine residues in RNA are targeted, and result in modifications including methylation and deamination. Such modified residues as N-6-methyl-adenosine (m6A) and inosine, respectively, have been associated with cardiovascular diseases, and contribute to disease pathologies. The Ischemic Heart Disease Epitranscriptomics and Biomarkers (IHD-EPITRAN) study aims to provide a more comprehensive understanding to their nature and role in cardiovascular pathology. The study hypothesis is that pathological features of IHD are mirrored in the blood epitranscriptome. The IHD-EPITRAN study focuses on m6A and A-to-I modifications of RNA. Patients are recruited from four cohorts: (I) patients with IHD and myocardial infarction undergoing urgent revascularization; (II) patients with stable IHD undergoing coronary artery bypass grafting; (III) controls without coronary obstructions undergoing valve replacement due to aortic stenosis and (IV) controls with healthy coronaries verified by computed tomography. The abundance and distribution of m6A and A-to-I modifications in blood RNA are charted by quantitative and qualitative methods. Selected other modified nucleosides as well as IHD candidate protein and metabolic biomarkers are measured for reference. The results of the IHD-EPITRAN study can be expected to enable identification of epitranscriptomic IHD biomarker candidates and potential drug targets.

Outcome of valve sparing root replacement for diverse indications.

OBJECTIVES: We report the mid-term outcomes of valve-sparing aortic root replacement (VSRR) in a cohort including patients with bicuspid aortic valve (BAV), connective tissue disorder (CTD), aortic dissection (AD), and congenital heart disease (CHD). Design. From 2005 to 2017, 174 patients underwent VSRR with the reimplantation technique. The mean age was 46 ± 14 years. The mean follow-up time was 4.8 ± 2.8 years. The indication for operation was aortic aneurysm for 127 (73%), aortic insufficiency (AI) for 38 (22%), and AD for 9 patients (5%). Preoperatively, 53 patients (31%) had ≥ moderate AI. BAV, CTD (Marfan or Loyes-Dietz), previous Ross procedure, or CHD was present in 57 (33%), 28 (16%), 7 (4%) and 12 patients (7%), respectively. Concomitant aortic valve repair was performed for 103 patients (59%). Results. Thirty-day mortality was zero. Four patients underwent aortic valve replacement (AVR) during follow-up. Kaplan-Meier estimates for survival, freedom from AVR, and freedom from ≥ moderate AI or reoperation were 96, 98, and 97% at 5 years. There was no difference in survival, freedom from AVR, or freedom from ≥ moderate AI or reoperation in patients with and without BAV, CTD, leaflet repair, or preoperative ≥ moderate AI. In Cox regression analysis, BAV, CTD, aortic valve repair, preoperative ≥ moderate AI, or aortic dimension were not risk factors for reoperation or valve dysfunction. Conclusions. Mid-term outcomes of VSRR for patients with diverse indications in terms of survival, reoperation rate, and valve dysfunction rate were excellent in a center with a limited annual volume of VSSR.

Right atrial and SVC infiltrating mass-the entity of infiltrating lipoma.

BACKGROUND: Cardiac lipomas are rare benign primary cardiac tumours primarily composed of mature adipocytes. They are usually well defined, encapsulated masses, but rarely demonstrate malignant characteristics by infiltrating the myocardium. This causes diagnostic uncertainty as it becomes a priority to rule out primary malignant cardiac tumours such as sarcoma which often carry a poor prognosis. CASE REPORT: A 61 year old female presenting with chest pain was found to have an infiltrating right atrial hypertrophic mass. Mutli-disciplinary team (MDT) discussions along with the presence of symptoms and likelihood of malignancy led to the recommendations for surgery. Intraoperatively this involved the right pulmonary veins and superior vena cava (SVC). The mass was resected with good margins and reconstruction of the right atrium, pulmonary veins and SVC was done using porcine pericardial patch. The patient made a good postoperative recovery and was discharged home in sinus rhythm with no significant valvular lesions. This was further confirmed at 6 month follow up. Final histology was that of infiltrating lipoma. CONCLUSIONS: In this rare case of infiltrating cardiac lipoma in a relatively young patient, the diagnostic uncertainty despite multimodal imaging meant surgery was indicated due to the high suspicion of cancer. Even in benign cases, fatty infiltration can lead to conduction defects and embolisation. Technical difficulties in sectioning these specimens is caused by intra-tumour variability and current recommendations are for excision biopsy, for best characterisation. The management of these patients requires an MDT with Cardiac surgery being a safe approach providing definitive management.

Echocardiographic predictors of reoperation for subaortic stenosis in children and adults.

OBJECTIVES: Subaortic stenosis (SAS) can present as various types of obstruction of the left ventricular outflow tract (LVOT) below the level of the aortic valve. Even though corrective surgery has been identified as the most effective treatment, SAS more frequently reoccurs requiring reoperation in a significant proportion of the patients. Previous studies have focused on predictors of recurrence in various subgroups of patients with SAS, but rarely in the overall population of patients with SAS. The aim of this study was to determine the predictors of recurrence of SAS after initial corrective surgery. METHODS: Patients from the database of the Congenital Cardiology Department of the University Hospital of Southampton with significant SAS requiring corrective surgery were included in the study. Data retrieved were obtained and used to determine the predictors of SAS recurrence after the initial corrective surgery. RESULTS: Eighty-two patients (paediatric, n = 72 and adult, n = 10) who underwent initial successful resection were included in the analysis. Thirty patients required reoperation for recurrent SAS. These were significantly younger (median age 3.0 vs 6.7 years, P = 0.002). The recurrence of SAS was more common in patients with an interrupted aortic arch (23.3% vs 3.8%, P = 0.010) and unfavourable left ventricle geometry (43.3% vs 7.6%, P < 0.001), with steeper aortoseptal angle (131.0° ± 8.7° vs 136.1° ± 8.6°, P = 0.030), shorter distance between the point of obstruction of the LVOT and the aortic valve annulus in systole and diastole (median 4.30 vs 5.90 mm, P = 0.003 and 3.65 vs 4.95 mm, P = 0.006, respectively) and in those who had higher residual peak and mean LVOT gradients postoperatively (29.3 ± 16.0 vs 19.8 ± 10.7 mmHg, P = 0.006 and 15.9 ± 8.3 vs 10.1 ± 5.8 mmHg, P = 0.002, respectively). Overall, the presence of an interrupted aortic arch [odds ratio (OR) 10.34, 95% confidence interval (CI) 1.46-73.25; P < 0.019] and unfavourable left ventricle geometry (OR 10.42, 95% CI 1.86-58.39; P < 0.008) could independently predict reoperation for SAS after initial successful resection. CONCLUSIONS: Patients who have initial corrective surgery for SAS at a younger age, unfavourable left ventricle geometry, an interrupted aortic arch and higher early postoperative LVOT gradients are more likely to have recurrent SAS requiring reoperation.

Hybrid transcatheter tricuspid valve-in-valve placement of an Edwards Sapien XT valve in palliated hypoplastic left heart syndrome.

Tricuspid regurgitation in the context of hypoplastic left heart syndrome (HLHS) carries a significant burden of both mortality and morbidity. We report successful hybrid trans-atrial implantation of an Edwards Sapien XT valve into a dysfunctional bioprosthetic valve in the tricuspid position of a 9-year-old patient with HLHS. There was an immediate fall in the Fontan pressures combined with significant clinical improvement and the valve continues to function well nearly 3 years post implant. To the best of our knowledge, this is the first described implantation of a transcatheter valve in this specific position and anatomy.

Primary surgical repair of tetralogy of Fallot at under three months of age.

Background Classical management of small infants with tetralogy of Fallot has involved placement of a Blalock-Taussig shunt followed later by complete repair, rather than primary complete repair which is the strategy adopted in larger infants. Some advantages of early complete repair compared to a staged strategy have been shown. We sought to review our institutional outcomes. Methods Patients under 3-months old undergoing complete surgical repair of tetralogy of Fallot in our institution between 2005 and 2015 were retrospectively reviewed and compared with an older control group matched by anatomical diagnosis and outflow tract intervention. Results Fourteen index cases (group A) and 14 controls (group B) were identified. At surgery, the median age was 43 days and weight 4.2 kg in group A, and 130 days and 6.1 kg in group B. Nine of 14 in group A were admitted for surgery as emergencies compared to none in group B. Peak inotrope score (22.3 vs. 12.8, p = 0.02) and intensive care unit stay (4.4 vs. 2.6 days, p = 0.02) were higher in group A. Bypass and crossclamp times, duration of intubation, and total length of stay did not differ. Conclusions We conclude that although babies undergoing early repair of tetralogy of Fallot have an increased need for intensive care support in the early postoperative period, the total length of stay is not prolonged despite more emergency admissions. As it is known that early repair may reduce long-term morbidity, we propose consideration of earlier complete repair of tetralogy of Fallot.

Open and closed distal anastomosis for acute type A aortic dissection repair.

OBJECTIVES: The current consensus favours an open distal anastomosis for aortic dissection repair. A small number of experiences have compared early and long-term outcomes between closed and open distal anastomosis in the setting of acute aortic dissection. METHODS: We reviewed our experience in 204 patients who underwent repair of spontaneous acute type A aortic dissection between January 2000 and December 2013. Open distal repair was performed in 109 patients, whereas 95 patients received a closed anastomosis. The clinical presentation, anatomical characteristics of aortic dissection, surgical techniques and the outcomes were analysed in the overall population and in the subgroup of patients (n = 100; open = 39, closed = 61) with Type 1 DeBakey dissection and a proximal intimal tear. Twenty-six preoperative and operative variables were studied to determine their impact on hospital mortality and postoperative neurological deficits. Imaging follow-up was available in 83 patients. RESULTS: A more extensive involvement of the aortic arch characterized the open repair group. No differences in terms of mortality, morbidity and survival rates were observed between the two groups of patients. Open repair with cerebral perfusion was associated with a better neurological outcome. Patients who underwent an open distal anastomosis showed a significant higher rate of complete thrombosis of the false lumen. CONCLUSIONS: An open repair does not increase the risk of early mortality and positively affect the evolution of the false lumen in distal unresected aortic segments. The use of cerebral perfusion reduces the risk of perioperative neurological injury.

Valve-sparing aortic root replacement in adult patients previously operated for congenital heart defects: an initial experience.

OBJECTIVES: To review our experience with valve-sparing aortic root replacement (VSARR) in adult patients after the previous surgery for congenital heart disease. METHODS: From August 2008 to February 2014, 11 patients (mean age: 30.5 ± 7.7 years), previously operated for various congenital cardiac defects, mostly conotruncal lesions, underwent VSARR for progressive aortic root dilatation. Five patients had also developed moderate, and 1 patient severe aortic valve (AoV) insufficiency. All aortic root procedures were performed by the same operating surgeon in two institutions. The mean interval between the initial procedure and aortic root surgery was 25.7 ± 7.7 years. At the time of reoperation, the mean aortic root diameter was 53.4 ± 6.2 mm. The reimplantation technique (David procedure) was used in all patients. Seven patients underwent concomitant procedures. The median follow-up was 32 months (range 12-78 months). RESULTS: No mortality occurred in hospital or during the follow-up period. Ten patients remain in NYHA functional class I; 1 patient with a single-ventricle circulation is in NYHA functional class II. During the immediate postoperative period, 2 patients underwent drainage of a pericardial collection and 1 patient required renal replacement therapy with eventual full recovery of renal function. No other reoperations or reinterventions were needed during the follow-up period. The most recent echocardiogram in 10 patients have demonstrated no or trivial AoV insufficiency. One patient, however, with severe aortic insufficiency preoperatively, developed mild AoV regurgitation shortly after the operation, which has progressed to moderate regurgitation 1 year following the operation. CONCLUSIONS: Analysis of our experience in a small group of patients confirms that valve-sparing aortic root surgery can be safely performed in adult congenital patients presenting with progressive aortic root dilatation following their previous surgery. Although we have analysed patients with different original congenital cardiac lesions and the follow-up period is not long, we can conclude that the use of the reimplantation technique (David procedure) have resulted in overall satisfactory AoV function at follow-up. Longer follow-up with larger series will be needed, however, before firm conclusions can be drawn.

Acute type A aortic dissection repair in elderly patients.

OBJECTIVES: We evaluated our experience in acute type A aortic dissection (ATAAD) repair in elderly patients. The role of clinical presentation and surgical strategies in determining patients' outcome was further assessed. METHODS: A retrospective analysis of patients over 75 years who underwent emergency repair of ATAAD at our institution during 2000-13 was performed. Forty-five patients (mean age = 79 ± 3 years; 26 females) were identified. Aortic dissection was complicated in 17 (37%) patients with new neurological deficit (n = 5), cardiac tamponade (n = 12), acute myocardial infarction (n = 5) and acute renal failure (n = 2). The ascending aorta was replaced in all patients and hypothermic circulatory arrest was employed in 22 patients. The aortic replacement needed extension to the hemiarch in 11 patients and the aortic valve was replaced in 9 patients; in 3 cases, full root replacement was performed. RESULTS: The in-hospital mortality rate was 15% (n = 7). Preoperative acute neurological deficit was the only independent risk factor for mortality (P = 0.03). Age >80 years old per se was not associated with a poor outcome. Surgical strategies and extension of aortic wall resection did not affect the operative mortality. The postoperative course was complicated in 23 (52%) patients. During the median follow-up of 57 months, there were 4 late deaths. The cumulative 1-, 5- and 8-year survival rates were 82, 76 and 67%, respectively. CONCLUSIONS: Emergency surgical repair of ATAAD in elderly patients resulted in an acceptable early mortality rate and satisfactory intermediate survival. Preoperative acute neurological deficit predicts a worse outcome. Advanced age alone should not be considered as a contraindication to AAD repair.

Hemodynamic performance of Trifecta: single-center experience of 400 patients.

OBJECTIVE: To evaluate postoperative hemodynamic gradients and early outcomes of aortic valve replacement with the Trifecta bioprosthesis. METHODS: Between 2011 and 2013, 400 patients underwent aortic valve replacement with a Trifecta bioprosthesis. Gradients were calculated by transthoracic echocardiography before discharge. Data were collected retrospectively; patients with postoperative severe left ventricular dysfunction or > mild mitral regurgitation were excluded. RESULTS: The mean age was 75.9 ± 8.5 years, 197 (49.25%) patients were male, and 140 (35%) were >80-years old. Concomitant procedures were performed in 207 (51.75%) patients, and 30 (7.5%) had redo procedures. Supraannular aortoplasty with bovine pericardium was necessary in 25 (6.25%) cases. Hospital mortality was 2.75% (11 patients). Postoperative peak and mean gradients were 21.7 ± 9.3 and 11.1 ± 4.3 mm Hg for 19-mm valves (n = 29); 19.5 ± 7 and 9.7 ± 3.6 mm Hg for 21-mm valves (n = 158); 17.3 ± 6.6 and 8.7 ± 3.2 mm Hg for 23-mm valves (n = 134); 15.1 ± 6.1 and 7.8 ± 3.3 mm Hg for 25-mm valves (n = 56); 13.2 ± 3.7 and 6.9 ± 2.6 mm Hg for 27-mm valves (n = 11). Nine patients had trivial and one had mild transvalvular regurgitation. Mean follow-up was 1 ± 0.62 years; no patient required reoperation. Kaplan-Meier survival at 1 and 2 years was 94.3% ± 1.3% and 93.7% ± 1.4%. CONCLUSION: Early postoperative gradients are low after Trifecta implantation. Significant transvalvular regurgitation was not observed, but the incidence of supraannular aortoplasty may be increased.

Expanding indications for valve-sparing aortic root reconstruction: early and midterm results.

BACKGROUND: Valve-sparing aortic root reconstruction (VSRR) is an accepted method to treat patients with aortic root dilation. The role of the VSRR is less well defined for patients with bicuspid aortic valve, severe aortic valve insufficiency, congenital heart defects, and type A aortic dissection. We studied the clinical outcome of patients who underwent VSRR for expanded indications. METHODS: Seventy-eight patients underwent VSRR between the 2005 and 2012. Seventy-two patients (92%) underwent reimplantation and 6 patients (8%) were operated on with the remodeling technique. The mean age was 51 ± 12 years (range 24 to 73). For 71 patients (91%), the operation was elective, and for 7 (9%; all with type A aortic dissection), on an emergency basis. Preoperatively, the degree of aortic insufficiency was graded as 2+ or greater for 27 patients (35%). Connective tissue disorder (Marfan or Loeys-Dietz), bicuspid aortic valve, or congenital heart disease was present in 15 (19%), 15 (19%), and 7 patients (9%), respectively. Concomitant aortic valve leaflet repair was performed for 39 patients (50%). The mean follow-up time was 2.4 ± 1.7 years (range, 0.1 to 7.0). RESULTS: Thirty-day mortality was zero. The rate of postoperative complications was low: stroke 3%, renal failure 3%, prosthesis infection 1%, and low cardiac output syndrome 1%. Survival was 100% at 1 year and 97% at 5 years. Freedom from recurrent aortic valve insufficiency (≥2+) during the follow-up was 94%. CONCLUSIONS: The midterm results of VSRR in terms of survival, freedom from recurrent aortic valve insufficiency, and the need for reoperation are excellent, even for high-risk patients.

Long-term outcomes in octogenarians following aortic valve replacement.

BACKGROUND: The aging of the population has resulted in an increasing number of elderly patients undergoing cardiac operations. We reviewed our experience in patients over the age of 80 undergoing primary aortic valve replacement (AVR) with or without CABG. METHODS: Between 2000 and 2008, 345 patients (226 male) ≥80 years underwent primary AVR in our unit. The notes of these patients were retrospectively reviewed and follow-up information was obtained from their general practitioners. They had a mean age of 82.9 ± 2.3 years and a median logistic EuroSCORE of 13.4 (IQR 9.4, 19.1). Isolated AVR was performed in 161 patients (45.5%), and 184 (51.6%) patients underwent combined AVR and CABG. A quality of life questionnaire was sent to all survivors. RESULTS: Hospital mortality occurred in 17 patients (4.9%), which was significantly lower than the mortality predicted by logistic EuroSCORE (16.2%, p < 0.01). Hospital mortality was comparable between patients undergoing isolated AVR and those undergoing additional CABG (4.3% vs. 5.4%, respectively). Actuarial survival at one and five years was 90.1 ± 1.6% and 77.2 ± 2.9%, respectively. There was a 62% response on the questionnaire showing 70% of the patients were NYHA I and 83.7% were satisfied with the operation outcome. CONCLUSIONS: AVR can be undertaken with excellent results in octogenarians and the current risk is significantly lower than what is predicted with conventional risk-scoring systems. Patients with advanced age should not necessarily be excluded from being candidates for AVR.

An unusual complication from hemostatic packing to control bleeding during cervical mediastinoscopy and an off-pump approach for the later exploration of the superior vena caval injury.

Cervical mediastinoscopy has been widely used and is considered a safe method for the histologic diagnosis and staging of many conditions. Hemorrhage still remains one of the main possible complications, and hemostasis is usually achieved without any further surgical intervention. We present a previously unreported complication of absorbable hemostatic gauze packing, which led to superior vena caval injury and multiple pulmonary emboli, necessitating further surgical repair with the use of a veno-venous shunt.