Pulmonary venous obstruction after extracardiac total cavopulmonary connection in right atrial isomerism.

BACKGROUND: Patients with functional single ventricle and right atrial isomerism (RAI) often have multiform cardiac pulmonary venous (PV) connection, which could be a risk factor for pulmonary venous obstruction (PVO) after extracardiac total cavopulmonary connection (EC-TCPC) owing to compression of the conduit. OBJECTIVE: To investigate the anatomical risk factors for PVO after EC-TCPC in RAI. METHODS: Twenty-nine patients with RAI without extracardiac total anomalous pulmonary venous connection were enrolled. No patients had PVO before EC-TCPC. A total of 14 and 15 patients had PV orifices ipsilateral and contralateral to the extracardiac conduit, respectively. The former 14 patients were assigned to two groups based on development of PVO after EC-TCPC (groups O and N). The pre- and post-operative cardiac morphologies and their relationship with the conduit were compared. RESULTS: After the EC-TCPC, the pressure gradients between the atrium and the PV were 5.0 ± 2.5 and 0.44 ± 0.2 mmHg in groups O and N, respectively (p < 0.01); however, the pressure gradients in the left and right PVs were not significantly different, suggesting stenosis of the common PV orifice. The ratio of the horizontal distance from the vertebrae to the PV orifice and to the lateral edge of the atrium was significantly higher (0.38 ± 0.2 vs. 0.17 ± 0.1; p = 0.04) and the orifice was smaller (8.9 ± 2.0 vs. 15 ± 4.7 mm; p < 0.01) in group O than in group N. CONCLUSION: In cases with ipsilateral locations of the conduit and PV orifice, small size and more lateral location of the PV orifice may be preoperative risk factors for development of PVO.

Post-Extubation Inhaled Nitric Oxide Therapy via High-Flow Nasal Cannula After Fontan Procedure.

In 2014, our hospital introduced inhaled nitric oxide (iNO) therapy combined with high-flow nasal cannula (HFNC) oxygen therapy after extubation following the Fontan procedure in patients with unstable hemodynamics. We report the benefits of HFNC-iNO therapy in these patients. This was a single-center, retrospective review of 38 patients who underwent the Fontan procedure between January 2010 and June 2016, and required iNO therapy before extubation. The patients were divided into two groups: patients in Epoch 1 (n = 24) were treated between January 2010 and December 2013, receiving only iNO therapy; patients in Epoch 2 (n = 14) were treated between January 2014 and June 2016, receiving iNO therapy and additional HFNC-iNO therapy after extubation. There were no significant differences between Epoch 1 and 2 regarding preoperative cardiac function, age at surgery, body weight, initial diagnosis (hypoplastic left heart syndrome, 4 vs. 2; total anomalous pulmonary venous return, 5 vs. 4; heterotaxy, 7 vs. 8), intraoperative fluid balance, or central venous pressure upon admission to the intensive care unit. Epoch 2 had a significantly shorter duration of postoperative intubation [7.2 (3.7-49) vs. 3.5 (3.0-4.6) hours, p = 0.033], pleural drainage [23 (13-34) vs. 9.5 (8.3-18) days, p = 0.007], and postoperative hospitalization [36 (29-49) vs. 27 (22-36) days, p = 0.017]. Two patients in Epoch 1 (8.3%), but none in Epoch 2, required re-intubation. Our results suggest that HFNC-iNO therapy reduces the duration of postoperative intubation, pleural drainage, and hospitalization.

Antibiotic Prophylaxis for Open Chest Management After Pediatric Cardiac Surgery.

OBJECTIVES: Although open chest management optimizes hemodynamics after cardiac surgery, it increases postoperative infections and leads to increased mortality. Despite the importance of antibiotic prophylaxis during open chest management, no specific recommendations exist. We aimed to compare the occurrence rates of bloodstream infection and surgical site infection between the different prophylactic antibiotic regimens for open chest management after pediatric cardiac surgery. DESIGN: Retrospective, single-center, observational study. SETTING: PICU at a tertiary children's hospital. PATIENTS: Consecutive patients younger than or equal to 18 years old with open chest management after cardiac surgery followed by delayed sternal closure, between January 2012 and June 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We compared the composite occurrence rate of postoperative bloodstream infection and surgical site infection within 30 days after cardiac surgery between three prophylactic antibiotic regimens: 1) cefazolin, 2) cefazolin + vancomycin, and 3) vancomycin + meropenem. In 63 pediatric cardiac surgeries with open chest management, 17 bloodstream infections, and 12 surgical site infections were identified postoperatively. The composite occurrence rates of bloodstream infection and surgical site infection were 10 of 15 (67%), 10 of 19 (53%), and nine of 29 (31%) in the cefazolin, cefazolin + vancomycin, and vancomycin + meropenem regimens, respectively (p = 0.07). After adjusting for age, open chest management duration, extracorporeal membrane oxygenation use, and nasal methicillin-resistant Staphylococcus aureus colonization in multivariable analysis, there was no significant difference between the cefazolin and the cefazolin + vancomycin regimens (p = 0.19), while the vancomycin + meropenem regimen had a lower occurrence rate of bloodstream infection and surgical site infection than the cefazolin regimen (odds ratio, 0.0885; 95% CI, 0.0176-0.446; p = 0.003). CONCLUSIONS: In this study, a lower occurrence rate of postoperative bloodstream infection and surgical site infection was observed among patients with broad-spectrum antibiotic regimen after pediatric cardiac surgery with open chest management. Further studies, ideally randomized controlled studies investigating the efficacy of broad-spectrum antibiotics and their complications, are warranted before routine implementation of broad-spectrum prophylactic antibiotic regimen.

Hepatic vein redirection to improve pulmonary arteriovenous malformations after Fontan completion in patients with absence of inferior vena cava and hemiazygos continuation.

Pulmonary arteriovenous malformation is an important complication after Fontan completion in patients with univentricular circulation. Lack of hepatic venous flow in a pulmonary artery has been identified as a cause of pulmonary arteriovenous malformation. We report our experience with a case of redirection of the hepatic vein to the hemiazygos vein using a conduit via left thoracotomy and median sternotomy for the correction of unequal distribution of hepatic venous flow in the pulmonary arteries. The pulmonary arteriovenous malformation improved, leading to increased arterial saturation levels.

Left ventricular function after a Fontan operation in patients with pulmonary atresia with an intact ventricular septum.

OBJECTIVES: In patients with pulmonary atresia with an intact ventricular septum (PA/IVS), there are no reports about the impact of sinusoidal communication (SC) on left ventricular (LV) performance after a Fontan operation; therefore, the purpose of this study was to fill this gap in the literature. METHODS: We performed a single-centre, retrospective study of 46 patients with PA/IVS. Nineteen patients who underwent the Fontan procedure were enrolled and divided into 2 groups: those with SC (SC group) and those without SC (N group). The 2 groups were compared in terms of postoperative cardiac function. RESULTS: Thirteen patients were in the SC group (4 patients with right ventricle-dependent coronary circulation) and 6, in the N group. Although 2 of the patients with right ventricular (RV)-dependent coronary circulation showed focal asynergy of the LV wall, others showed no findings of myocardial infarction. The patients' preoperative age and age during the observation period after the Fontan operation did not differ. There was no difference in LV ejection fraction, cardiac index and RV and pulmonary artery pressure before and after the Fontan operation. Preoperative RV volume in the N group was greater than that in the SC group. Brain natriuretic peptide levels were higher in the SC group after the Fontan operation. On ventricular efficacy analysis, contractility was lower (1.8 ± 0.32 vs 2.5 ± 0.40 mmHg/ml/m2, P = 0.001) and ventricular efficacy was worse (1.0 ± 0.15 vs 0.86 ± 0.11, P = 0.046) in the SC group during the postoperative period. CONCLUSIONS: Compared to patients with PA/IVS without SC, patients with PA/IVS with SC had a low cardiac contractility and decreased cardiac output efficiency after the Fontan procedure.

Bilateral Pulmonary Artery Banding in Ebstein's Anomaly With Circular Shunting.

We present a case of critical Ebstein's anomaly with circular shunting, diagnosed in utero. The fetal cardiothoracic area ratio was elevated; tricuspid regurgitation and pulmonary regurgitation worsened with fetal hydrops. At 35 weeks 6 days of gestation, elective caesarean section delivery was performed. Planned bilateral pulmonary artery banding and pulmonary trunk ligation were performed as a palliative operation 4 hours after birth, with the infant in a stable condition. At age 5 days, we performed the Starnes operation. The postoperative course was uneventful and a bidirectional Glenn operation was performed at age 4 months.

Supra-Annular Mitral Valve Replacement in an Infant With Infective Endocarditis.

We report the case of a female neonate born at 40 weeks' gestation with no known risk factors. On postnatal day 27, she was transferred to the pediatric intensive care unit and intubated for cardiorespiratory failure; she was diagnosed with group B streptococcus infective endocarditis. Mitral valve vegetations did not improve with antibiotic therapy. Mitral valve surgery was performed on postnatal day 36. A mechanical mitral valve with short-segment polytetrafluoroethylene skirt was inserted into the supra-annular position; gentian violet was applied to the sewing ring and skirt. She was discharged from the hospital after 6 weeks with good cardiac function and normal sinus rhythm.

Fontan operation with bilateral conduits for the treatment of pulmonary arteriovenous fistulas in a patient with polysplenia after reconstruction of a non-confluent pulmonary artery.

We describe a case involving a successful Fontan procedure for the treatment of pulmonary arteriovenous fistulas in a patient with polysplenia. The patient was diagnosed with bilateral superior vena cavae, interrupted inferior vena cava with azygos connection, wide separate hepatic veins, and central pulmonary artery stenosis following repair of a non-confluent pulmonary artery. Bilateral extracardiac conduits were used to connect each hepatic vein to the pulmonary artery, opposite each superior vena cava. Clinical improvement in the pulmonary arteriovenous fistulas was observed within 4 months after surgery.

Right atrial aneurysm with downward displacement of the anterior leaflet that resembled Ebstein's anomaly.

A 13-year-old boy presented with right atrial aneurysm and downward displacement of the anterior leaflet in the tricuspid valve into the right ventricle, without tricuspid valve regurgitation. Paroxysmal atrial flutter was caused by an abnormal electrical re-entry circuit, which could not be treated using catheter radiofrequency ablation. Therefore, the patient underwent surgical ablation and resection of the enlarged right atrial wall. The anterior leaflet of the tricuspid valve was plastered and displaced downward into the right ventricle, which resembled Ebstein's anomaly. Pathological evaluation revealed a thin wall that contained fibrous tissue with lipomatous degeneration and few muscular elements. No postoperative arrhythmia was observed.

Risk Factors for Semilunar Valve Insufficiency After the Damus-Kaye-Stansel Procedure.

BACKGROUND: Cardiac function and survival after the Damus-Kaye-Stansel (DKS) procedure are encouraging, but only limited data exist related to postprocedural semilunar valve function. We investigated postprocedural midterm to long-term outcomes and changes in semilunar valve function and identified risk factors of semilunar valve function deterioration. METHODS: Between 1996 and 2012, 63 patients with a single functional ventricle underwent the DKS procedure. Of them, 50 had previously undergone pulmonary artery banding. Cardiac function was measured by catheter examination 5.3 months (interquartile range, 2.6 to 9.7) preoperatively and 1.2 years (interquartile range, 1.0 to 1.4) postoperatively. Echocardiographic examination of the semilunar valve was performed concurrently with the catheter examination and at the last follow-up (5.3 years [interquartile range, 3.1 to 9.2] postoperatively). RESULTS: The overall survival rate at 1, 5, and 10 years postoperatively was 0.97, 0.92, and 0.89, respectively. Aortic and pulmonary valve regurgitation grade 1 year postoperatively and at the last follow-up increased compared with the preoperative grade. There was no significant difference between regurgitation at 1 year and the last follow-up. The duration from pulmonary artery banding to the DKS procedure was longer in the group with at least mild regurgitation (n = 6) than in the group with less than mild regurgitation (n = 54). CONCLUSIONS: Although the duration from pulmonary artery banding to the DKS procedure was associated with postoperative regurgitation, neither postprocedural aortic nor pulmonary valve regurgitation increased over time, and there were no deleterious effects on the clinical conditions.

Pulmonary artery rupture after bilateral pulmonary artery banding in a neonate with Loeys-Dietz syndrome and an interrupted aortic arch complex: report of a case.

Loeys-Dietz syndrome (LDS) is a recognized connective tissue disorder characterized by progressive aortic aneurysm and dissection. Patients are at high risk of aortic dissection or rupture at an early age, but to our knowledge, surgery on the great arteries has never been attempted in the neonatal period. We report a case of LDS with dilated pulmonary arteries and an interrupted aortic arch complex in a neonate. We performed bilateral pulmonary artery banding, but 12 days after the procedure, the infant died of rupture of the distal portion of the banding sites following massive dilatation.

Right pulmonary artery obstruction is a long-term complication of aortopulmonary window repair.

The early outcomes of transaortic patch repair closure for aortopulmonary window are satisfactory, but the lifelong fate of the aorta and pulmonary artery remains unknown. We describe a 40-year-old patient with right pulmonary artery occlusion accompanied by aneurysmal dilation of the ascending aorta 38 years after transaortic repair of an aortopulmonary window. Operative findings revealed patch shrinkage and thrombotic occlusion of the right pulmonary artery. The dilated ascending aorta firmly adhered to the right pulmonary artery. After pulmonary artery thrombectomy, the right pulmonary artery was reconstructed and the dilated ascending aorta was replaced.

Use of the Damus-Kaye-Stansel procedure prevents increased ventricular strain in Fontan candidates.

OBJECTIVES: In Fontan candidates, we have recently been aggressively performing the Damus-Kaye-Stansel procedure (DKS) to prevent increased afterload on the systemic ventricle. The present study investigated the efficacy of the DKS procedure in terms of the ventricular function following a Fontan operation. METHODS: Patients undergoing a Fontan operation were divided into three groups: DKS performed at the time of the bidirectional Glenn or Fontan operation (DKS group, n = 25); DKS not performed at any stage due to mild pulmonary stenosis (PS) (PS group, n = 23) and DKS not performed due to pulmonary atresia (PA) or severe PS (PA group, n = 24). Ventricular function, afterload on the systemic ventricle and atrioventricular valve regurgitation were compared between groups. RESULTS: Cardiac catheterization before a Glenn or Fontan operation and at 1-year following the Fontan revealed significantly decreased ventricular end-diastolic pressure in the DKS group. The ventricular ejection fraction was significantly deteriorated in the PS group. Effective arterial elastance, as an index of total ventricular afterload, was increased after a Fontan in all groups, with a substantial increase in the PS group. As an index of ventricular mechanical efficiency, ventriculoarterial coupling was significantly increased only in the PS group. Cardiac ultrasonography revealed atrioventricular valve regurgitation above grade 3 persisting in many patients from the PS group. Semilunar valve function after DKS operation did not progress to moderate or worse in any patients. CONCLUSIONS: The proactive performance of the DKS procedure prevents increased ventricular afterload, avoiding deterioration of cardiac function and contributing to improved long-term results following a Fontan operation.

Acute myocardial infarction caused by a floating thrombus in the ascending aorta: A role of CD34-positive endothelial cells.

A 49-year-old woman was transferred to our hospital with acute-onset chest pain. Her electrocardiogram showed complete atrioventricular block and bradycardia with ST-segment elevation in the inferior leads, and she presented with cardiogenic shock. She was diagnosed with inferior acute myocardial infarction (AMI), and subsequent emergency cardiac catheterization was performed. Selective coronary angiography showed neither stenosis nor obstruction in any of the coronary arteries. Left ventriculography showed a large floating object located on the ascending aortic wall above the ostium of the right coronary artery (RCA). Chest enhanced computed tomography confirmed the floating object in the ascending aorta. These findings suggested that the floating object was associated with the RCA occlusion. To remove the floating object, emergency surgery was performed. The floating object was a large thrombus derived from the localized atheromatous plaque in the ascending aorta. Specialized immunostaining for surface antigen CD34 revealed that regenerated endothelial cells were present on the erosion, along the stalk, and on the floating thrombus. These findings indicate that the CD34-positive endothelial precursor cells strayed into the surface and/or inside of the thrombus, and consequently the floating thrombus supported by these regenerated endothelial cells occluded the RCA, causing AMI. .

Continuous hemodiafiltration in children after cardiac surgery.

Acute renal failure (ARF) after congenital cardiac surgery remains a serious complication and leading cause of morbidity and mortality. Continuous hemodiafiltration (CHDF) is presently accepted for pediatric applications. We retrospectively evaluated the effects of CHDF against ARF after congenital cardiac surgery at our hospital. We analyzed data from seven patients aged 23 days to 9 years and weighing 1.7-22.4 kg requiring dialysis therapy using CHDF after congenital cardiac surgery between April 2002 and January 2009. One patient who died could not be weaned from extracorporeal membrane oxygenation support and another died of multiple organ failure. Renal function recovered to normal in the other five (71%) patients. Treatment by CHDF lasted from 14 to 680 h and net ultrafiltration was 3.5 ± 1.4 mL/kg/h. Serum creatinine and urea concentrations were, respectively, 2.3 ± 1.6 and 43.7 ± 17.0 mg/dL before, and 0.5 ± 0.2 and 13.5 ± 8.1 mg/dL, after CHDF (P < 0.05). Thrombocytopenia developed in all patients, and platelet concentrates (0.76 ± 0.7 mL/kg/h) were infused during CHDF. Hypotension developed after changing the CHDF set in one patient. We suggest that CHDF is an effective alternative strategy for treating renal dysfunction after congenital cardiac surgery.

Early and long-term outcome of total arch replacement using selective cerebral perfusion.

BACKGROUND: The aim of this study was to analyze mortality and morbidity of patients undergoing total arch replacement. METHODS: We analyzed data from 122 patients who underwent total arch replacement. Total arch replacement was performed using selective cerebral perfusion, with an open distal anastomosis using a trifurcated coated graft. Univariate and multivariate analysis were performed concerning postoperative neurologic dysfunction and early mortality. Long-term mortality was estimated by the Kaplan-Meier method. RESULTS: Permanent and temporary neurologic dysfunction were 4.1% (5 cases) and 5.7% (7 cases), respectively. Hospital mortality was 8.2% (10 cases). There was no significant difference in hospital mortality or neurologic dysfunction between the two groups divided by age less than or greater than 70 years old, emergency versus elective surgery, and dissection versus true aneurysm. After multivariate analysis, independent predictors for hospital mortality were found to be infection, operation time longer than 10 hours, and EuroSCORE greater than 15; whereas those for neurologic dysfunction were operation time longer than 10 hours and ischemic heart disease. Overall long-term survival was 80.4% at 5 years and 58.9% at 10 years. CONCLUSIONS: The early and long-term outcomes of total arch replacement using selective cerebral perfusion and appropriate establishment of cardiopulmonary bypass were satisfactory.

Bidirectional cavopulmonary shunt with additional pulmonary blood flow.

There are some controversies regarding the usefulness of leaving additional pulmonary blood flow when establishing a bidirectional cavopulmonary shunt. From April 2002 to September 2008, 13 patients (mean age, 24 +/- 16 months) underwent a bidirectional cavopulmonary shunt procedure with fine adjustment of additional pulmonary blood flow, as an intermediate step before the Fontan operation. There were no hospital deaths. Modified Blalock-Taussig shunts were left during the bidirectional cavopulmonary shunt operation in 7 patients, and pulmonary bands were tightened in 4. The main pulmonary artery with a previous pulmonary band was left open in one case. Oxygen saturation increased from 74.5% +/- 7.4% to 84.6% +/- 1.9% after the operation, cardiothoracic ratio decreased from 55.9% +/- 6.1% to 53.2% +/- 3.4%, Left ventricular end-diastolic pressure decreased from 11.0 +/- 2.6 to 7.8 +/- 3.0 mm Hg, and mean pulmonary arterial pressure from 14.7 +/- 7.5 to 10.2 +/- 3.1 mm Hg. Pulmonary artery index did not change significantly. In our experience, additional pulmonary blood flow with adjustment in each patient at the time of shunt construction was an excellent temporary palliation prior to the Fontan operation.

Extracorporeal membrane oxygenation circulatory support after congenital cardiac surgery.

Extracorporeal membrane oxygenation (ECMO) is widely used for circulatory support in pediatric cardiac patients with low cardiac output and hypoxemia after cardiac surgery. We evaluated retrospectively, the efficacy of postoperative ECMO support following congenital cardiac surgery in our hospital. From April 2002 to February 2008, seven patients (median age 30 months) received postoperative mechanical support. Three had complete repair including Fontan circulation and four had palliative repair. In four patients, ECMO was initiated in the operating room, in three patients in the intensive care unit, postoperatively. Of the seven patients, one died on ECMO (support withdrawn), one died shortly after ECMO was discontinued, and five were successfully weaned and survived to hospital discharge. One of the survivors died 4 months after the operation due to pneumonia and septic shock. The mean duration of ECMO support was 121 hours (in survivors). During ECMO, the major complication was bleeding, despite adequate control of activated clotting time. We conclude that ECMO support for heart failure and respiratory insufficiency after congenital cardiac surgery was effective, and the result of ECMO support for respiratory insufficiency was better than for heart failure.

[Thymic carcinoid tumor with Cushing syndrome].

A case of a 71-year-old male with ectopic adrenocorticotropic polypeptide (ACTH)-producing thymic carcinoid tumor presenting Cushing's syndrome was reported. This patient had symptoms of fatigue and a polyposia for 2 years before a mediastinal tumor was detected. Chest computed tomography (CT) scan demonstrated an anterior mediastinal mass, and serum ACTH and cortisol level revealed very high. Secretion of cortisol was not inhibited in an 8-mg dexamethazone suppression test. We diagnosed ectopic ACTH-producing tumor, and performed complete excision of the thymus including thymic tumor. Histologically, the tumor demonstrated typical carcinoid with the positivity of ACTH immunostaining. After the operation, ACTH and cortisol levels were reduced and the clinical symptoms were improved rapidly. We have concluded that it is important to control serum perioperative cortisol level for the prevension of morbidity.