Clinical Prognosis of Vocal Cord Paralysis After Cardiothoracic Surgery in Infants.

We aimed to clarify the long-term outcomes and prognosis of vocal cord paralysis (VCP) after cardiothoracic surgery in infants as well as the usefulness of laryngeal ultrasound (LUS) as screening for VCP. Overall, 967 infants aged 1-year-old or younger who underwent cardiothoracic surgery between 2008 and 2022 were included in this study. We divided the patients into two groups based on the period on whether they underwent screening without or with LUS and compared the incidence of VCP between the groups. There were no differences in the patients' preoperative characteristics between the two periods, whereas the incidence of VCP was significantly higher in period 2 than in period 1 (11.0% vs. 3.2%, p < 0.0001). The incidence of VCP among the procedures, including aortic arch repair, was > 50% and significantly increased from period 1 to period 2. The sensitivity and specificity of LUS was 87% and 90%, respectively. Symptoms of VCP improved in 92% of patients. Repeated flexible laryngoscopy revealed that the residual rate of VCP was 68%, 52%, and 48% at 6, 12, and 24 months, respectively. In conclusion, symptoms of postoperative VCP improved in most cases; however, paralysis persisted in half of the patients. As a screening method, LUS is useful for evaluating postoperative VCP. A more accurate understanding of VCP is needed to improve postoperative outcomes.

Usefulness of 18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in the Diagnosis of Infective Endocarditis in Patients With Adult Congenital Heart Disease.

BACKGROUND: Infective endocarditis (IE) in patients with adult congenital heart disease (ACHD) remains a diagnostic challenge due to difficulties in detecting endocardial lesions by echocardiography. 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) has shown good diagnostic performance in prosthetic valve IE. This study aimed to assess its additional diagnostic value in ACHD-associated IE and to characterize its advantages.Methods and Results:Overall, 22 patients with ACHD and clinical suspicion of IE were retrospectively studied. 18F-FDG PET/CT was performed in addition to conventional assessment based on the modified Duke criteria. The final IE diagnosis was determined by an expert team during a 3-month clinical course, resulting in 18 patients diagnosed with IE. Seven patients (39%) were diagnosed with definite IE only by initial echocardiography. An 18F-FDG PET/CT assessment revealed endocardial involvement in the other 9 patients, resulting in the diagnosis of definite IE in 16 in total (88%). Right-sided endocardial lesions were more common (n=12, 67%) but rarely identified by echocardiography, whereas 18F-FDG PET/CT revealed right-sided lesions in 9 patients. A negative 18F-FDG PET/CT (n=7, 39%) assessment was associated with a native valve IE (71% vs. 0%). In 4 patients who were identified with not-IE, neither echocardiography nor 18F-FDG PET/CT detected any suspicious cardiac involvement. CONCLUSIONS: In the diagnosis of ACHD-associated IE, characterized by right-sided IE, 18F-FDG PET/CT assessment should be useful.

Successful papillary muscle approximation for severe mitral regurgitation via apical cuff hole in HeartMate 3 implantation via left anterior thoracotomy.

We herein report a case of successful papillary muscle approximation for severe mitral regurgitation and HeartMate 3 left ventricular assist device implantation via left anterior thoracotomy in a 39-year-old man diagnosed with dilated cardiomyopathy. He underwent papillary muscle approximation in the mitral valve for severe functional regurgitation via the apical cuff hole. The postoperative course was uneventful. Echocardiography revealed that mitral regurgitation has disappeared. He was awaiting heart transplantation while working.

Single-stage hybrid total arch replacement for extended arch aneurysms.

OBJECTIVE: The therapeutic strategy for extended aortic arch aneurysms remains controversial and has changed substantially since thoracic endovascular aortic repair was introduced. We applied single-stage hybrid (s-hybrid) total arch replacement (TAR), which involved ascending aorta replacement and debranching of arch vessels, consecutively performed with thoracic endovascular aortic repair for extended arch aneurysms. The aim of this study was to investigate the short-term results of s-hybrid TAR and to clarify the benefit of this method. METHODS: We reviewed the operative results of 62 patients who underwent elective s-hybrid TAR or conventional TAR (c-TAR) through the median approach from 2008 to 2017. We used the s-hybrid approach in 15 patients and the c-TAR approach in 47 patients. In both groups, axillary arterial perfusion and selective antegrade cerebral perfusion under moderate hypothermia were applied for brain protection. We compared the perioperative outcomes of the two groups. RESULTS: We completed s-hybrid TAR in all 15 patients with extended aneurysms. The s-hybrid group required shorter times for myocardial ischemia, selective antegrade cerebral perfusion, and circulatory arrest of the lower body compared with the c-TAR group. The patients with complicated recurrent laryngeal nerve palsy and long ventilation support times were fewer in the s-hybrid group. No patient had substantial endoleaks or permanent paraplegia. The in-hospital mortality rates were 6.7% in the s-hybrid group and 0% in the c-TAR group. CONCLUSIONS: The s-hybrid TAR has the same or better perioperative outcomes compared with the c-TAR approach. For extended aneurysms, this technique could resolve the problem of respiratory failure induced by left thoracotomy and also resolve the problem of rupture during the waiting period in staged surgery.

Jarvik 2000 axial flow ventricular assist device in right single ventricle after Fontan operation.

We present a case of successful ventricular assist device support in a 13-year-old female diagnosed with right single ventricle, asplenia, dextrocardia, who had undergone a Fontan operation at 4 years old in an associated children hospital. She underwent placement of Jarvik 2000 axial flow ventricular assist device to the morphologic right ventricle which worked as systemic ventricle. The postoperative course was not eventful. She was waiting for heart transplantation attending high school 3 years after implantation.

High incidence of ductal closure or narrowing at birth in patients with right ventricular outflow tract obstruction with normal orientation of the ductus arteriosus.

BACKGROUND: Ductal patency is mandatory to manage patients with ductal-dependent pulmonary circulation. The aim of this study is to elucidate the morphological and haemodynamic features of ductus arteriosus with right ventricular outflow tract obstruction, and investigate the appropriate perinatal management.Patients and methodsPatients with prenatal diagnosis of right ventricular outflow tract obstruction at our institution between 2010 and 2015 were included in the study. Reverse orientation of the ductus arteriosus is defined as an inferior angle of 90°. We retrospectively reviewed the shape and flow pattern of ductus arteriosus and the clinical characteristics of the cases. RESULTS: A total of 39 patients were enrolled. The shape was divided into normal orientation (n=15) and reverse orientation (n=24) of the ductus arteriosus. There was no significant difference in the type of oxygen saturation at birth and age at shunt operation between both the groups. However, the median narrowest diameter of ductus arteriosus in the normal orientation group was significantly smaller than that in the reverse orientation group (2.0 [1.0-5.4] versus 3.0 [1.3-4.4] mm, p<0.05). In two patients of the normal orientation group, ductus arteriosus had closed at birth, and one of whom died because of severe cyanosis. CONCLUSIONS: Normal orientation pattern might have high incidence of an early narrowing or closure of ductus arteriosus at birth. The critical patients need careful evaluation by repeated foetal echocardiography and further maternal interventions.

[Congenitally Corrected Transposition of the Great Arteries( TGA) in Adult].

OBJECTIVES: In patients with congenitally corrected transposition of the great arteries( cc-TGA), the morphological right ventricle (RV) sustains the systemic circulation and is frequently associated with progressive tricuspid regurgitation and declining RV function. METHODS: Thirty-four patients with cc-TGA and systemic RV were enrolled in this retrospective study to examine long-term outcome. RESULTS: Age was averaged 38.5 years. Associated cardiac anomaly included ventricular septal defect (VSD) and pulmonary stenosis in 18, VSD in 4. Twelve had no associated cardiac defect. The physiologic repair was performed in 22 patients at the mean age of 7.8 years old. Reoperation was frequent including tricuspid valve surgery in 11 and left ventricle( LV)-pulmonary artery( PA) conduit replacement in 10. Five patients needed pacemaker implantation due to heart block. Aortic insufficiency was a relatively common finding and aortic valve replacement was performed in 4. All but 2 patients were better than New York Heart Association (NYHA) class Ⅱ. One needed implantable ventricular assist device implantation and the other underwent heart transplantation. CONCLUSIONS: Long-term outcome for patients with cc-TGA was satisfactory. Frequent reoperations including tricuspid valve surgery and LV-PA conduit replacement were required. Further careful follow-up will be needed to detect the progression of RV dysfunction and tricuspid and aortic insufficiency.

Long-Term Comparison of Three Types of Aortic St. Jude Medical Mechanical Prosthesis in Japanese Patients.

BACKGROUND: The long-term results achieved with aortic St. Jude Medical (SJM) mechanical prostheses in various age groups of Japanese patients have not been previously compared or reported. METHODS AND RESULTS: Since 1981, a total of 240 SJM valves were implanted in 79 patients using the Standard model, in 58 patients with the Hemodynamic Plus model, and in 103 patients with the Regent model for aortic valve replacement (AVR). Follow-up was completed for 2,397 patient-years in 97.5% of the patients, among whom the effect of age was compared, and the subjects were divided into younger (<65 years) and older (≥65 years) groups. Hospital mortality rate was 2.5%. No structural valve deterioration was observed during the follow-up period. In addition, no significant differences were observed in long-term survival between the 3 models. In contrast, significantly better rates of freedom from all-cause death (P<0.0001), valve-related death (P=0.0018) and valve-related morbidity (P=0.0021), including bleeding events (P=0.0007), were observed in the younger group (n=157, 50.6±1.0 years old) than in the older group (n=83, 72.5±0.7 years old). CONCLUSIONS: All types of SJM valve used for single AVR achieved satisfactory early and long-term results in each age group even 25 years after surgery. When selecting this prosthesis for elderly patients, however, relatively worse performance may be expected compared with that observed in younger patients.

Long-term results of aortic valve replacement with mechanical prosthesis or carpentier-edwards perimount bioprosthesis in Japanese patients according to age.

BACKGROUND: The long-term results of aortic valve replacement (AVR; n=737) with bileaflet mechanical prosthesis (MP) or Carpentier-Edwards Perimount bioprostheses (BP) were evaluated in different age groups. METHODS AND RESULTS: Since 1981, a total of 737 prostheses (424 bileaflet MP vs. 313 BP) were implanted for AVR in 278 patients aged ≥70 years (79 MP vs. 199 BP), in 191 patients aged 60-69 years (128 MP vs. 63 BP) and in 268 patients aged <60 years (217 MP vs. 51 BP). Follow-up was completed for 6,523 patient-years in 98.5% of cases. Among the patients ≥70 years, both the actuarial survival rate (P=0.0434) and freedom from valve-related morbidity (P=0.0205) were better in the BP group than in the MP group without any difference in occurrence of structural valve deterioration in both groups. Among the patients aged 60-69, anticoagulant-related complications occurred less often in the BP group (P=0.0134) without any difference in long-term survival. Among the patients aged <60, long-term survival was significantly better in the MP group, whereas freedom from anticoagulant-related events did not differ. CONCLUSIONS: The use of BP is suitable in patients aged ≥70 years, while the use of bileaflet MP is preferable in patients aged <60 years. Among patients aged 60-69 years, the use of BP is acceptable because of the lower incidence of anticoagulant-related events and the equivalent long-term survival.

[Surgical strategy and the results for hypoplastic left heart syndrome].

OBJECTIVE: We retrospectively reviewed our surgical results in patients with hypoplastic left heart syndrome( HLHS) to investigate the influence of surgical strategy on outcome. METHODS: Seventy-seven patients with classic HLHS were involved in this study. For the initial palliation, 23 patients underwent Norwood operation with modified Blalock-Taussig (BT) shunt, 23 patients underwent Norwood operation with right ventricle to pulmonary artery( RV-PA) shunt and 31 patients underwent bilateral pulmonary artery banding (BPAB). Surgical results, freedom from Fontan operation, hemodynamic data and incidence of complication after Fontan operation were compared between the 3 groups. RESULTS: BPAB group had more preoperative risk factors than Norwood group. Total actuarial survival was 62.4% at 1 year and 58.2% at 3 and 5 years, and there was no difference between the groups. Freedom from Fontan completion was 59.4% at 3 years and 48.7% at 5 years without difference between the groups. The incidence of intervention for pulmonary artery stenosis was higher in Norwood with RV-PA shunt group(52.9%). Hemodynamic data obtained by cardiac catheterization were similar in the 3 groups, however, end-systolic elastance, which represents ventricular contractility, was lower in Norwood with RV-PA group. CONCLUSIONS: Surgical results of Norwood with BT shunt and Norwood with RV-PA shunt were comparably satisfactory, however, there was possible concern of reduced ventricular contractility in RV-PA shunt group. BPAB was a effective and useful initial palliation in high risk cases.

How do we treat severely prolapsed true ectopia cordis?

Ectopia cordis is an extremely rare condition where the heart displaces outside the thoracic cavity. Treating this, especially when accompanied by congenital heart diseases and severe protrusion, is challenging. Here, we present a case of successful treatment involving intracardiac repair and delayed sternal closure using local skin flaps.

Successful repair of infracardiac total anomalous pulmonary venous connection with nonconfluent bilateral pulmonary veins.

We report a rare case of an infracardiac-type total anomalous pulmonary venous connection with nonconfluent bilateral pulmonary veins in a patient diagnosed with heterotaxy syndrome with right atrial isomerism, mitral valve atresia, a single atrium, and double-outlet right ventricle. On the fourth day of life, the patient underwent successful repair using a sutureless technique. Two years after the surgery, the patient remained well without any signs of pulmonary venous obstruction.

[An adult case of tricuspid valve endocarditis with ventricular septal defect; report of a case].

A 50-year-old man was admitted to hospital because of an elevated fever. He had been diagnosed with ventricular septal defect in his childhood, but surgery had not been recommended. An echocardiogram showed vegetations on the tricuspid valve, severe tricuspid regurgitation and perimembranous ventricular septal defect. He was diagnosed with infective endocarditis( IE) and treated with antibiotics and diuretics. Five serious dental caries, which had probably caused IE, were found and extracted before surgery. After 4 weeks of medical treatment, we performed tricuspid valve repair and closed the ventricular septal defect. The postoperative course was uneventful. He has been free from any complication for over 3 years.

Regression of oesophageal varices in total anomalous pulmonary venous connection.

The coexisting of oesophageal varices with total anomalous pulmonary venous connection is extremely rare but contains a potential leading to a lethal haemorrhage. The fate of the oesophageal varices after total anomalous pulmonary vein connection repair remains largely unknown. We herein report a case with infracardiac type total anomalous pulmonary venous connection with remarkable oesophageal varices. In the present case, of note, the oesophageal varices were completely regressed after total anomalous pulmonary venous connection repair without any intervention. This case might help a surgical team reduce the hesitation to repair the total anomalous pulmonary venous connection regardless of oesophageal varices, a potentially fatal condition.

Valve-Sparing Reimplantation for Right Coronary Artery Compression After Fontan Procedure.

An incidental finding of right coronary artery compression between the dilated aortic root and the sternum was detected on follow-up cardiac catheterization after Fontan completion for tricuspid atresia with transposition of the great arteries. Although this 31-year-old man had no subjective symptoms, an exercise stress test and perfusion scan revealed ischemic changes in the inferior region. Valve-sparing aortic root replacement using the reimplantation technique proved to be an effective approach for this condition. The possibility of coronary artery compression associated with aortic root dilatation should be considered in adult patients with congenital heart disease.

[Influence of congenital heart defects on outcomes of noncardiac surgery in neonates].

We reviewed our experience to examine the outcomes of noncardiac surgery in neonates with congenital cardiac defects and assess future changes in the management of noncardiac surgery in neonates for whom new cardiac surgical management strategies will develop based on current progress in prenatal diagnosis. Forty-five neonates with congenital cardiac defects underwent noncardiac surgery in our institution. Subsequent cardiac surgery was performed in 26 of those neonates. No patient underwent cardiac surgery before noncardiac surgery. However, rescue neonatal cardiac surgery, which is performed within 24 h after birth, has become more common because of the progress in prenatal diagnosis. Under these circumstances in neonatal cardiac surgery, the timing or approach of cardiac and noncardiac surgery should be reconsidered in the next few years. We emphasize the importance of cooperation among relevant specialists in the management of neonates with multiple disorders to increase the benefits offered by developments in prenatal diagnosis.

A case of tricuspid atresia with pulmonary valve absence coexisting coronary-right ventricular fistula.

Tricuspid atresia with pulmonary valve absence is a rare malformation characterized by left ventricular outflow obstruction due to asymmetrical ventricular septal hypertrophy and associated with a poor prognosis. Coexisting coronary-right ventricular fistula is rarely described. We encountered a case of tricuspid atresia with pulmonary valve absence coexisting coronary-right ventricle fistula that successfully underwent Fontan completion. Right ventricle dilatation due to coronary-right ventricle fistula was observed in addition to mass like ventricular septal hypertrophy protruding into the left ventricular outflow tract. Right ventricle reduction and prevention of progressive left ventricular outflow obstruction were achieved by closure of the coronary-right ventricle fistula closure and plication of the right ventricle with the bidirectional Glenn procedure.

Long-term function of Hydrofit as hemostatic sealant: abridged republication.

The newly-designed hemostatic sealant, Hydrofit, was developed in Japan and consists of a urethane-based polymer without blood products. By applying Hydrofit gel to an anastomosis site on the aorta, water contact initiates a chemical change in the forming elastomer which adheres rapidly and tightly. We experienced an extirpation of Hydrofit gel that had been applied 4 years and 8 months previously in a 42-year-old female who underwent aortic valve replacement and graft replacement of the ascending aorta. The Hydrofit left around the aortic graft suture line was without infection and functioned very well as an elastic sealant for a long period.

Ascending aortic extension to enlarge the retroaortic space in children after the Norwood procedure.

Left pulmonary artery stenosis is a well-recognized complication following the Norwood procedure. We herein report two cases in which ascending aortic extension was performed to enlarge the retroaortic space in children with left pulmonary artery stenosis after the Norwood procedure. We used graft interposition in the ascending aorta to increase the retroaortic apace and concomitantly performed extended left pulmonary artery reconstruction. This procedure obtains a more balanced distribution of the pulmonary blood flow, which is crucial to achieve good Fontan circulation.

Pulmonary Valve Replacement via Left Thoracotomy as an Alternative to Resternotomy.

Most patients with repaired tetralogy of Fallot (TOF) survive to adulthood and suffer from residual right ventricular pathology, mostly pulmonary regurgitation. Pulmonary valve replacement (PVR) is a procedure of choice to alleviate right ventricular dilatation and pulmonary regurgitation. Resternotomy is the standard approach for PVR in patients who have undergone TOF repair. However, these patients require multiple reoperations during their lifetime. We performed minimally invasive redo PVR through left mini-thoratocomy in 2 patients who had previously undergone TOF repair through sternotomy.