Young infants with symptomatic tetralogy of Fallot: Shunt or primary repair?

Objectives: The optimal treatment strategy for symptomatic young infants with tetralogy of Fallot (TOF) is unclear. We sought to compare the outcomes of staged repair (SR) (shunt palliation followed by second-stage complete repair) versus primary repair (PR) at 2 institutions that have exclusively adopted each strategy. Methods: We performed propensity score-matched comparison of 143 infants under 4 months of age who underwent shunt palliation at one institution between 1993 and 2021 with 122 infants who underwent PR between 2004 and 2018 at another institution. The primary outcome was mortality. Secondary outcomes were postoperative complications, durations of perioperative support and hospital stays, and reinterventions. Median follow-up was 8.3 years (interquartile range, 8.1-13.4 years). Results: After the initial procedure, hospital mortality (shunt, 2.8% vs PR, 2.5%; P = .86) and 10-year survival (shunt, 95%; 95% confidence interval [CI], 90%-98% vs PR, 90%; 95% CI, 81%-95%; P = .65) were similar. The SR group had a greater risk of early reinterventions but similar rates of late reinterventions. Propensity score matching yielded 57 well-balanced pairs. In the matched cohort, the SR group had similar freedom from reintervention (55%; 95% CI, 39%-68% vs 59%; 95% CI, 43%-71%; P = .85) and greater survival (98%; 95% CI, 88%-99.8% vs 85%; 95% CI, 69%-93%; P = .02) at 10 years, as the result of more noncardiac-related mortalities in the PR group. Conclusions: In symptomatic young infants with TOF operated at 2 institutions with exclusive treatment protocols, the SR strategy was associated with similar cardiac-related mortality and reinterventions as the PR strategy at medium-term follow-up.

Survival benefits of the wait-and-grow approach in small babies (≤2000 g) requiring heart surgery.

Objective: The best approach to minimize the observed higher mortality of newborn infants with low birth weight who require congenital heart surgery is unclear. This retrospective study was designed to review outcomes of newborn infants weighing <2000 g who have undergone cardiovascular surgery to identify patient parameters and clinical strategies for care associated with higher survival. Methods: A retrospective chart review of 103 patients who underwent cardiovascular surgery from 2010 to 2021 who were identified as having low birth weight (≤2000 g). Patients who underwent only patent ductus arteriosus ligation or weighing >3500 g at surgery were excluded. Results: Median age was 24 days and weight at the time of surgery was 1920 g. Twenty-six (25%) operative mortalities were recorded. Median follow-up period was 2.7 years. The 1- and 3-year overall Kaplan-Meier survival estimate was 72.4% ± 4.5% and 69.1% ± 4.6%. The 1-year survival of patients who had a weight increase >300 g from birth to surgery was far superior to the survival of those who did not achieve such a weight gain (81.4% ± 5.6% vs 64.0% ± 6.7%; log-rank P = .04). By multivariable Cox-hazard regression analysis, the independent predictor of 1-year mortality was genetic syndrome (hazard ratio, 3.54; 95% CI, 1.67-7.82; P < .001), whereas following a strategy of increasing weight from birth to surgery resulted in lower mortality (hazard ratio, 0.49; 95% CI, 0.24-0.90; P = .02). Conclusions: A strategy of wait and grow for newborn infants with very low birth weight requiring heart surgery results in better survival than immediate surgery provided that the patient's condition allows for this waiting period.

Repair of the complete atrioventricular septal defect-impact of postoperative moderate or more regurgitation.

OBJECTIVES: To study the risk factors for mortality, moderate or more left atrioventricular valve regurgitation (LAVVR) and reoperation after the surgical repair of complete atrioventricular septal defect (cAVSD) in a single centre. METHODS: The current study is a retrospective review of patients who underwent surgical repair of cAVSD between 2000 and 2021. Patients with unbalanced ventricles not amenable to biventricular repair, double outlet right ventricle and malpositioned great arteries were excluded. The clinical predictors of outcome for end points were analysed with univariate and multivariable Cox regression analysis or Fine-Gray modelling for competing risks. Time-dependent end points were estimated using the Kaplan-Meier curve analysis and cumulative incidence curves. RESULTS: The median follow-up time was 2.3 years. Among 220 consecutive patients were 10 (4.6%) operative and 21 late mortalities (9.6%). A total of 26 patients were identified to have immediate postoperative moderate or more regurgitation and 10 of them ultimately died. By multivariable analysis prematurity and having more than moderate regurgitation immediately after the operation were identified as predictors of overall mortality (P = 0.003, P = 0.012). Five- and ten-year survival rates were lower for patients with immediate postoperative moderate or more LAVVR {51.9% [confidence interval (CI): 27.5-71.7%]} when compared to patients without moderate or more regurgitation [93.2% (CI: 87.1-96.4%) and 91.3% (CI: 83.6-95.5%)]. CONCLUSIONS: The patients who undergo cAVSD repair remain subjected to a heavy burden of disease related to postoperative residual LAVVR. Immediate postoperative moderate or more LAVVR contributes significantly to overall mortality. Whether a second run of bypass can decrease this observed mortality should be investigated.

A uniform strategy of primary repair of tetralogy of Fallot: Transventricular approach results in low reoperation rate in the first decade.

OBJECTIVES: To review outcomes after a uniform strategy of transventricular repair of tetralogy of Fallot. METHODS: A total of 244 consecutive patients underwent transventricular primary repair of tetralogy of Fallot from 2004 to 2019. Median age at operation was 71 days; 57 (23%) patients were premature; 57 (23%) patients had low birth weight (<2.5 kg), and 40 (16%) had genetic syndromes. The diameter of pulmonary valve annulus, right pulmonary artery (PA), and left PA were 6.0 ± 1.8 mm (z score, -1.7 ± 1.3), 4.3 ± 1.4 mm (z score, -0.9 ± 1.2) and 4.1 ± 1.5 mm (z score, -0.5 ± 1.3). RESULTS: Three (1.2%) operative deaths were recorded. Ninety patients (37%) underwent transannular patching. Postoperative echocardiographic peak right ventricular outflow tract gradient decreased from 72 ± 27 mm Hg to 21 ± 16 mm Hg. Median intensive care unit and hospital stay were 3 and 7 days. The survival rate at 10 years was 94.6% ± 1.8%. Reintervention was required 86 times (55 catheter interventions) in 56 patients following tetralogy of Fallot repair. The freedom from all-cause reintervention rate at 10 years was 70.5% ± 3.6%. Cyanotic spells (hazard ratio, 2.14; 95% CI, 1.22-3.90; P < .01) and smaller pulmonary valve annulus z score (hazard ratio, 1.26; 95% CI, 1.01-1.59; P = .04) were associated with increasing risk of all reinterventions. Freedom from redo surgery for right ventricular outflow tract obstruction and right ventricular dilatation at 10 years were, respectively, 85.0% ± 3.1% and 98.7% ± 0.9%. Freedom from valve implantation was 96.7% ± 1.5% at 10 years. CONCLUSIONS: A uniform strategy of primary repair of tetralogy of Fallot through a transventricular approach resulted in low reoperation rate in the first decade. The need of pulmonary valve implantation was limited to <4% at 10 years.

Impact of time from symptom onset to operation on outcome of repair of acute type A aortic dissection with malperfusion.

OBJECTIVES: We analyzed patients with acute type A aortic dissection complicated by malperfusion syndrome to establish whether the timing of operative treatment and the location of malperfusion are factors in determining outcomes. METHODS: A total of 331 patients with acute type A aortic dissection were treated surgically between August 2003 and May 2019. Eighty-four patients (25%) presented with preoperative malperfusion syndrome. Fifty-eight patients with malperfusion syndrome (69%) were transferred to the operating room within 5 hours of the onset of symptoms (immediate repair); 26 patients (31%) were transferred after 5 hours (later repair). We analyzed the effects of immediate aortic repair on surgical outcomes. RESULTS: There was no significant difference in the early mortality rates between patients with immediate and later aortic repair, which were 20.0% (n = 11/58) and 26.9% (n = 7/19), respectively (P = .12). Preoperative coronary malperfusion was the only predictor of early mortality. The cumulative 5-year survivals of patients with malperfusion syndrome in the immediate and later repair groups were 76.7% and 45.4%, respectively. A significant difference was noted in the long-term outcomes between the 2 groups (P = .02). On multivariable Cox survival analysis, coronary malperfusion and shock on arrival were associated with increased long-term mortality (P < .01 and P = .04). Conducting surgery within 5 hours of the onset of symptoms was a significant predictor of favorable long-term outcome (P = .03). CONCLUSIONS: Although preoperative coronary malperfusion and shock on arrival worsened the long-term outcomes in patients undergoing aortic repair for acute type A aortic dissection with preoperative malperfusion syndrome, conducting an operation within 5 hours of the onset of symptoms significantly improved their long-term outcomes.

Mesenchymal Stromal Cell Delivery Via Cardiopulmonary Bypass Provides Neuroprotection in a Juvenile Porcine Model.

Oxidative/inflammatory stresses due to cardiopulmonary bypass (CPB) cause prolonged microglia activation and cortical dysmaturation, thereby contributing to neurodevelopmental impairments in children with congenital heart disease (CHD). This study found that delivery of mesenchymal stromal cells (MSCs) via CPB minimizes microglial activation and neuronal apoptosis, with subsequent improvement of cortical dysmaturation and behavioral alteration after neonatal cardiac surgery. Furthermore, transcriptomic analyses suggest that exosome-derived miRNAs may be the key drivers of suppressed apoptosis and STAT3-mediated microglial activation. Our findings demonstrate that MSC treatment during cardiac surgery has significant translational potential for improving cortical dysmaturation and neurological impairment in children with CHD.

Giant Patent Ductus Arteriosus Aneurysm Compressing the Esophagus.

It is extremely rare to observe aneurysmal changes in patients with patent ductus arteriosus (PDA), especially in adults. If left untreated, a PDA aneurysm can increase the risk of life-threatening complications, including rupture, dissection, esophageal fistula, and infection. Following is a description of successful surgical repair in a 55-year-old man with PDA aneurysm compressing the esophagus. Histologically, the aneurysmal wall showed mild thickening of the intima and media with hyperplastic smooth muscle cells, but no destructive changes were observed.

Cardiovascular surgery in very low birth weight (≤1500 g) neonates.

OBJECTIVES: Low birth weight and prematurity are known risks of increased morbidity and mortality with undergoing cardiovascular surgery. Our aim was to review the outcomes of very low birth weight (≤1500 g) patients who have undergone cardiovascular surgery. METHODS: A retrospective review was performed for 32 very low birth weight (≤1500 g) patients who underwent cardiovascular surgery from 2004 to 2021 in our institution. RESULTS: Fifteen patients weighting ≤1500 g at surgery (≤1500-g group) were compared to 17 patients born with a weigh of ≤1500 g and weighting between 1500 and 2500 g at surgery (>1500- to ≤2500-g group) in this study. In-hospital mortality was 33% (5/15) in the ≤1500-g group and 24% (4/17) in the >1500- to ≤2500-g group (P = 0.55). All patients with simple biventricular lesion survived following full repair. The occurrence of postoperative intracerebral haemorrhage was significantly higher in those operated at weight ≤1500 g than those weighting >1500 to ≤2500 g (40% vs 0%; P = 0.01). The 1- and 3-year survival rates were 66.0 ± 12.4% and 46.2 ± 14.8% in the ≤1500-g group and 76.5 ± 10.3% and 70.6 ± 11.1% in the >1500- to ≤2500-g group (log-rank P = 0.12). CONCLUSIONS: Cardiac surgery for a very low birth weight neonate resulted in a high early and late mortality. Early surgery is only acceptable for simple biventricular lesions if needed. Delayed surgery seems to provide better long-term outcomes in patients with complex lesions. Alternative strategies for neonatal cardiopulmonary bypass should be investigated in patients with complex biventricular and single ventricular lesions.

Excision of large right atrial myxoma through a right mini thoracotomy.

Cardiac myxoma is the most common primary benign cardiac tumor in adults and right atrial myxoma is a rare observation. We report a case of a 56-year-old woman who presented with dyspnea and diagnosed with a right atrial myxoma. Urgent operation through a right mini thoracotomy was done and myxoma was completely excised. Traditionally, median sternotomy with cardiopulmonary bypass is used for excision of cardiac myxoma. Excision through a mini thoracotomy for patients with right atrial myxoma appear to be safe, feasible and efficacious.

Impact of High-Resolution Epiaortic Ultrasonographic Imaging on Evaluating Aortic Wall Pathology.

There has been no definitive method, other than pathological findings, to identify the degeneration of the tunica media in the aortic wall (TM). We describe how high-resolution intraoperative epiaortic ultrasonographic imaging identifies changes in the TM of patients with aortic dissection. This method shows great promise in facilitating presymptomatic diagnoses of various aortic wall pathologies.

Retrospective study of thoracic endovascular aortic repair as a first-line treatment for traumatic blunt thoracic aortic injury.

OBJECTIVE: This study sought to confirm if thoracic endovascular aortic repair (TEVAR) was an appropriate therapeutic strategy for blunt thoracic aortic injury (BTAI). METHODS: Between 3/2005 and 12/2020, 104 patients with BTAI were brought to our hospital. The severity of each trauma case was evaluated using the Injury Severity Score (ISS); aortic injuries were classified as type I to IV according to Society for Vascular Surgery guidelines. Initial treatment was categorized into four groups: nonoperative management (NOM), open aortic repair (OAR), TEVAR, or emergency room thoracotomy/cardiopulmonary resuscitation (ERT/CPR). RESULTS: The patients' mean age and ISS were 56.7 ± 20.9 years and 48.3 ± 20.4, respectively. Type III or IV aortic injury were diagnosed in 82 patients. The breakdown of initial treatments was as follows: NOM for 28 patients, OAR for four, TEVAR for 47, and ERT/CPR for 25. The overall early mortality rate was 32.7%. Logistic regression analysis confirmed ISS > 50 and shock on admission as risk factors for early mortality. The cumulative survival rate of all patients was 61.2% at 5 years after treatment. After initial treatment, eight patients receiving TEVAR required OAR. The cumulative rate of freedom from reintervention using TEVAR at 5 years was higher in approved devices than in custom-made devices (96.0 vs. 56.3%, p = 0.011). CONCLUSIONS: Using TEVAR as an initial treatment for patients with BTAI is a reasonable approach. Patients with severe multiple traumas and shock on admission had poor early outcomes, and those treated with custom-made devices required significant rates of reintervention.

Acute Kidney Injury Affects Mid-Term Outcomes of Thoracoabdominal Aortic Aneurysms Repair.

The effect of acute kidney injury (AKI) on mid-term outcomes following thoracoabdominal aortic aneurysm (TAAA) repair is not well known. We hypothesized that postoperative AKI would reduce mid-term survival and aimed to analyze the effect of AKI on mid-term outcomes after TAAA repair. This retrospective study identified 294 consecutive TAAA repairs at Kobe University Hospital from October 1999 to March 2019. Patients with preexisting end-stage renal disease that required hemodialysis (n = 11) and patients who died intraoperatively (n = 2) were excluded. Finally, 281 patients were analyzed. AKI was defined according to Kidney Disease: Improving Global Outcomes guidelines (KDIGO) classification. Of the 281 patients, 178 (63.3%) developed AKI, of which 98 (34.9%) had mild, 34 (12.1%) had moderate, and 46 (16.4%) had severe AKI. Twenty-six patients (12.8%) required renal replacement therapy after surgery. Twenty-three in-hospital deaths (8.2%) were recorded, including 2 (0.7%) without AKI, 0 (0%) with mild AKI, 1 (0.4%) with moderate AKI, and 20 (7.1%) with severe AKI (p < .001). The 4-year survival was 91.9 ± 3.0% for no AKI, 91.3 ± 3.2% for mild AKI, 72.4 ± 8.5% for moderate AKI and 32.6 ± 7.4% for severe AKI (p < .001). Multivariable Cox-hazard regression analysis demonstrated that moderate and severe AKI, older age and emergency surgery were significant risk factors for mid-term survival. In patients undergoing TAAA repair, severe AKI was associated with an increase in in-hospital mortality and both moderate and severe AKI were negatively associated with mid-term survival. Preventing moderate/severe AKI may improve mid-term survival after TAAA repair.

Impending paradoxical embolism.

An impending paradoxical embolism (IPDE) is seldom observed in clinical practice. We report a case of IPDE in a 67-year-old female with severe dyspnea and hypotension, which was detected and successfully treated with emergent cardiac surgery. The optimal treatment is still controversial. We believe that emergent surgery always should be considered in patients with IPDE. .

Right ventricular outflow tract obstruction caused by sinus of Valsalva aneurysm.

Right ventricular outflow tract (RVOT) obstruction caused by sinus of Valsalva aneurysm is a rare observation. We describe a successful case of valve-sparing root replacement using reimplantation technique for RVOT obstruction by a large right coronary sinus of Valsalva aneurysm in a 76-year-old man. In the pathological examination, the elastic fibers of the medial layer were defective not only in the wall of the aneurysmal Valsalva sinus but also in the remaining two sinus walls. Our experience illustrated that valve-sparing root replacement can be an effective procedure in such a case.

Mid-term outcomes of valve-sparing root reimplantation with leaflet repair.

OBJECTIVES: Valve repair for aortic insufficiency (AI) requires a tailored surgical approach determined by the leaflet and aortic disease. In this study, we used a repair-oriented system for the classification of AI, and we elucidated long-term outcomes of aortic root reimplantation with this classification system. METHODS: From 1999 to 2018, a total of 197 patients underwent elective reimplantation (mean age: 52.7 ± 17.7 years; 80% male). The aortic valve was tricuspid in 143 patients, bicuspid in 51 patients and quadricuspid in 3 patients. A total of 93 patients had type I AI (aortic dilatation), 57 patients had type II AI (cusp prolapse) and 47 patients had type III AI (restrictive). In total, 104 of the 264 patients (39%) had more than 1 identified mechanism. RESULTS: In-hospital mortality was 0.5% (1/197). Mid-term follow-up (mean follow-up duration: 5.5 years) revealed a late mortality rate of 4.2% (9/197). Aortic valve reoperation was performed on 16 patients (8.0%). Rates of freedom from aortic valve replacement and freedom from aortic valve-related events at 10 years of follow-up were 87.0 ± 4.0% and 60.6 ± 6.0%, respectively; patients with type Ib AI (98.3 ± 1.7%; 80.7 ± 7.5%) had better outcomes than patients with type III AI (59.6 ± 15.6%; 42.2 ± 13.1%, P = 0.01). In patients with types II and III AI who had bicuspid aortic valves, rates of freedom from aortic valve-related events at 5 years of follow-up were 95.2 ± 4.7% and 71.7 ± 9.1%, respectively (P = 0.03). CONCLUSIONS: This repair-oriented system for classifying AI could help to predict the durable aortic valve repair techniques. Patient selection according to the classification is particularly important for long-term durability. CLINICAL TRIAL REGISTRATION NUMBER: B190050.

Thoracic Endovascular Aortic Repair for Ruptured Intercostal Patch Aneurysm Following Thoracoabdominal Aortic Aneurysm Repair.

Aneurysmal change of reconstructed intercostal arteries is believed to be a rare complication after thoracoabdominal aortic repair. To our knowledge, there is no guideline or randomized controlled trials regarding intercostal patch aneurysm management. Therefore, the optimal treatment is still controversial. We describe a successful case of emergent thoracic endovascular aortic repair for ruptured intercostal patch aneurysm in an 83-year-old man following thoracoabdominal aortic aneurysm repair. Our experience illustrated that gradual expansion of large blocks of aortic wall reconstruction should be closely monitored after primary thoracoabdominal aortic replacement.

A successful report of mitral valve repair for parachute-like mitral valve in adult.

A 65 year-old man was diagnosed with congestive heart failure secondary to severe mitral regurgitation resulting from a parachute-like asymmetrical mitral valve. We performed mitral annuloplasty and triangular resection of the thick tissue. The postoperative course was uneventful with no recurrence of mitral regurgitation.

Impact of shaggy aorta on outcomes of open thoracoabdominal aortic aneurysm repair.

BACKGROUND: The aim of this study was to evaluate the impact of diffuse aortic atherosclerosis-related thrombosis, or "shaggy aorta" on the outcomes of open thoracoabdominal aortic aneurysm repair (TAAA). METHODS: From October 1999 to March 2018, 251 patients underwent open TAAA repair using segmental-staged aortic clamping. Twenty-eight patients (11.2%) received emergent or urgent operations. Patients were classified into 3 groups: dissection aneurysm (139 patients, 55.4%), degenerative aneurysm without shaggy aorta (76 patients, 30.3%), and degenerative aneurysm with shaggy aorta (36 patients, 14.3%). Shaggy aorta was assessed using enhanced computed tomography and defined as patients with atheroma thickness ≥5 mm with irregular atheroma surface. Mean follow-up was 4.3 ± 4.1 years. RESULTS: Operative mortality was 8% (20 patients) and spinal cord injury occurred in 25 patients (10.0%), 16 of whom (6.4%) had permanent neurologic dysfunction. Operative mortality was significantly worse in patients with shaggy aorta (dissection: 2.2%, non-shaggy: 6.6%, and shaggy: 33.3%, P < .001) and shaggy aorta was a significant risk factor for spinal cord injury (dissection: 7.2%, non-shaggy: 6.6%, and shaggy: 27.8%, P < .003). Multivariable analysis demonstrated that shaggy aorta was a significant risk factor for composite outcome consisted of operative mortality, spinal cord injury, and acute renal failure (odds ratio, 4.78; 95% confidence interval, 1.91-12.3, P < .001). CONCLUSIONS: Preoperative enhanced computed tomography assessment of shaggy aorta could predict high-risk patients for open TAAA repair.

Mid-term outcomes of simultaneous mitral valve repair in patients with miral regurgitation and concomitant annulo-aortic ectasia.

OBJECTIVE: This study investigated the mid-term outcomes of simultaneous mitral valve repair in patients with mitral regurgitation (MR) and concomitant annulo-aortic ectasia. METHODS: The study included 26 patients with MR and annulo-aortic ectasia [mean age 46.0 ± 19.9 (10-86) years] who underwent simultaneous mitral valve repair between January 2007 and March 2018. Of these 26 patients, 11 (42.3%) were diagnosed with Marfan syndrome and 10 (38.5%) with Barlow's disease. All patients underwent complete ring annuloplasty; a semi-rigid ring was used in 14 (53.8%) and a semi-flexible ring (anterior-flexible) in 12 patients (46.2%). All patients underwent valve-sparing root replacement using the reimplantation technique. RESULTS: The overall 3-year survival rate was 95.7 ± 4.3%. The 3-year freedom from > moderate MR rate was 94.7 ± 5.1%, and the 3-year freedom from > moderate aortic regurgitation (AR) rate was 86.7 ± 7.3%. The 3-year freedom from reoperation rate was 100%. The 3-year freedom from > moderate MR rate was 100% in the semi-rigid ring group and 85.7 ± 13.2% in the semi-flexible ring group (log-rank test, p = 0.5371). The 3-year freedom from > moderate AR rate was 100% in the semi-rigid ring group and 72.9 ± 16.5% in the semi-flexible ring group (log-rank test, p = 0.0815). CONCLUSIONS: Simultaneous mitral valve repair in patients with MR and concomitant annulo-aortic ectasia showed favorable mid-term outcomes.