[Mitral and Aortic Valve Replacement and Tricuspid Valve Repair in a Patient with Metal Allergy].

We report a 63-year-old woman came to our hospital with exertional dyspnea, palpitations, and abdominal distention. Echocardiography showed mitral, aortic, and tricuspid valve insufficiency, for which surgery was indicated. Twenty-six years ago, during dental therapy, she was diagnosed with metal allergy. A patch test demonstrated allergic reactions to manganese, chromium, and zinc. The patient underwent mitral and aortic valve replacement with the On-X prosthetic heart valve, which is primarily made of titanium and devoid of the allergens. She also underwent tricuspid valve repair with a Contour 3D annuloplasty ring, which is made of titanium alloy. She manifested no allergic symptoms three years after surgery. This case elucidates the importance of history taking regarding metal allergy and identification of allergens by patch testing in patients undergoing cardiac surgery involving metal device implantation.

Late management of the aortic root after repair of tetralogy of Fallot: A European multicentre study.

OBJECTIVES: We sought to determine the indications, type, and outcomes of reoperations on the aortic root after repair of tetralogy of Fallot (TOF). METHODS: Eleven centers belonging to the European Congenital Heart Surgeons Association contributed to the data collection process. We included 36 patients who underwent surgical procedures on the aortic root, including surgery on the aortic valve and ascending aorta, between January 1975 and December 2017. Original diagnoses included TOF-pulmonary stenosis (n = 18) and TOF-pulmonary atresia (n = 18). The main indications for reoperation were aortic insufficiency (n = 19, 53%), aortic insufficiency and dilatation of the ascending aorta (n = 10, 28%), aortic root dilatation (n = 4, 11%), and ascending aorta dilatation (n = 3, 8%). RESULTS: The median age at reoperation was 30.4 years (interquartile range 20.3-45.3 years), and mechanical aortic valve replacement was the most common procedure performed. Five patients died early after reoperation (14%), and larger ascending aorta diameters were associated with early mortality (P = .04). The median age at the last follow-up was 41.4 years (interquartile range 24.5-51.6 years). Late death occurred in five patients (5/31, 16%). Most survivors (15/26, 58%) were asymptomatic at the last clinical examination (New York Heart Association, NYHA class I). The remaining patients were NYHA class II (n = 7) and III (n = 3). The most common symptoms were fatigue (n = 5), dyspnea (n = 4), and exercise intolerance (n = 3). CONCLUSIONS: Reoperations on the aortic root are infrequent but may become necessary late after TOF repair. The main indications for reoperation are aortic insufficiency, either isolated or associated with a dilatation of the ascending aorta. The surgical risk at reoperation was high and the presence of ascending aorta dilation is related to higher mortality.

[Midterm Outcomes and Therapeutic Effects of Total Cavopulmonary Connection (TCPC) Conversion].

Total cavopulmonary connection (TCPC) conversion prevents late complications after the atrio-pulmonary-Fontan procedure. However, the outcomes and effects of TCPC conversion are not fully known. We performed TCPC conversion in 31 patients (2004~2017). Concomitantly, we performed anti-arrhythmia surgery in 28 patients( 90%), atrioventricular valve surgery in 4, and pacemaker implantation in 2. There were no perioperative deaths, but one late death occurred due to protein-losing gastroenteropathy. Five-year survival was 96.8%. Eleven patients were readmitted for various reasons, including arrhythmia in 7 and heart failure in 1. The 5-year cardiac event-free rate was 67.7%. The cardiac index was significantly improved:1.58 l/min/m2 before to 3.57 l/min/m2 after surgery ( p=0.0075). Surgical and midterm outcomes of TCPC conversion were favorable. In the perioperative and late periods, therapeutic intervention was usually for atrial arrhythmia. This study demonstrated that TCPC conversion is an effective therapeutic procedure.

[Mid-aortic Syndrome Requiring Surgical Intervention during Infancy;Report of a Case].

Mid-aortic syndrome (MAS) is a very rare disease characterized by stenosis from the distal of the thoracic aorta to the abdominal aorta, in many case it is found as a result of hypertension and the like, and it needs surgical intervention in early childhood to adolescence. Here, we report a case of MAS which recognized prominent left ventricular myocardial hypertrophy from the early stage and needed surgical intervention in the infancy. We selected patch angioplasty using expanded polytetrafluoro ethylene( ePTFE) graft, and after surgery pressure gradient was disappeared.

Giant coronary artery aneurysm occluded completely by a thrombus.

A coronary artery aneurysm is an uncommon vascular disorder, and it can be a life-threatening disease when associated with rupture or an embolism. A 52-year-old man was found to have a 50-mm coronary artery aneurysm at the right coronary artery, and the aneurysm was completely occluded by a thrombus. He had no symptoms after arriving at our hospital, and his hemodynamics was stable. Therefore, initially, we administered anticoagulation therapy involving heparin. After therapy, the distal coronary artery was detected when the thrombus dissolved, and elective surgery was planned. Coronary artery bypass grafting, ligation of the inflow and outflow vessels, and resection of the aneurysm were performed. Early anticoagulation therapy and surgical aneurysm resection were effective for treating the completely occluded coronary artery aneurysm. We herein report this rare case of a giant coronary artery aneurysm occluded completely by a thrombus and treated successfully by anticoagulation therapy and surgical aneurysm resection.

[Association of lung volume with shunt size in Starnes' procedure in neonates with severe Ebstein's anomaly].

Our modification of Starnes' procedure reduces right ventricular volume using only "suture plication" to improve surgical outcomes. However, shunt size in the procedure varies widely between patients. As this may be related to small lung volume, we estimated lung volume in each patient using computed tomography (CT).Since 2007, we have performed Starnes' procedure in 4 patients. Preoperative cardiothoracic ratio was 89±4.5%. Age and body weight at operation were 4.3±2.6 days and 2.6±0.2 kg, respectively. Anatomic slices 3 mm thick were acquired in transverse planes by CT. Total lung volume was calculated by accumulating those slices. Total lung volume and lung volume/body weight were 97.2±34.1 ml, 36.8±11.5 ml/kg, respectively. In one patient, a 3 mm prosthetic graft was needed to place a clip to regulate blood flow. Another patient required an additional shunt. The patient with the smallest lung volume required treatment with an extracorporeal lung-assistance device. Chest X-rays of neonates with severe Ebstein's anomaly usually show a "wall-to-wall" heart. However, lung volume varies widely between patients. Estimation of lung volume using CT is useful. In patients with smaller lung volume, a larger shunt than usual may be required to obtain the necessary pulmonary blood flow.

Effect of procedural volume on the outcomes of congenital heart surgery in Japan.

OBJECTIVES: The present study developed a new risk model for congenital heart surgery in Japan and determined the relationship between hospital procedural volume and mortality using the developed model. METHODS: We analyzed 47,164 operations performed between 2013 and 2018 registered in the Japan Cardiovascular Surgery Database-Congenital and created a new risk model to predict the 90-day/in-hospital mortality using the Japanese congenital heart surgery mortality categories and patient characteristics. The observed/expected ratios of mortality were compared among 4 groups based on annual hospital procedural volume (group A [5539 procedures performed in 90 hospitals]: ≤50, group B [9322 procedures in 24 hospitals]: 51-100, group C [13,331 procedures in 21 hospitals]: 101-150, group D [18,972 procedures in 15 hospitals]: ≥151). RESULTS: The overall mortality rate was 2.64%. The new risk model using the surgical mortality category, age-weight categories, urgency, and preoperative mechanical ventilation and inotropic use achieved a c-index of 0.81. The observed/expected ratios based on the new risk model were 1.37 (95% confidence interval, 1.18-1.58), 1.21 (1.08-1.33), 1.04 (0.94-1.14), and 0.78 (0.71-0.86) in groups A, B, C, and D, respectively. In the per-procedure analysis, the observed/expected ratios of the Rastelli, coarctation complex repair, and arterial switch procedures in group A were all more than 3.0. CONCLUSIONS: The risk-adjusted mortality rate for low-volume hospitals was high for not only high-risk but also medium-risk procedures. Although the overall mortality rate for congenital heart surgeries is low in Japan, the observed volume-mortality relationship suggests potential for improvement in surgical outcomes.

Swiss-cheese ventricular septal defect closure by combination sandwich patch.

A 7-year-old boy with a history of neonatal pulmonary artery banding underwent almost complete closure of a sieve-like "Swiss-cheese" ventricular septal defect, using a combination sandwich patch technique through a right ventriculotomy. Although defects existed in the high-, mid-, and low-trabecular septa, a right ventriculotomy and division of the muscle trabeculations continuing the septal and moderator bands helped delineate the edges of the defects. Although patients with "Swiss-cheese" ventricular septal defects may be candidates for a Fontan operation conventionally, a combination patch technique could be considered the procedure of choice.

Long-term results of tricuspid reconstruction with pericardium in an infant.

We report the long-term clinical outcome of an 8-month-old infant who underwent tricuspid reconstruction using fresh autologous pericardium for severe tricuspid regurgitation due to defects in the leaflets of the tricuspid valve after ventricular septal defect closure and ablation. Ten years after surgery, the tricuspid function is good with mild regurgitation and mild stenosis. From the age of 8 months to 11 years, the patient's body weight and height increased fourfold and twofold, respectively. Thus, a fresh autologous pericardial patch could be the material of choice for tricuspid leaflet repair in pediatric patients.

Mid-term outcomes of Contegra implantation for the reconstruction of the right ventricular outflow tract to proximal branch pulmonary arteries: Japan multicentre study.

OBJECTIVES: To reveal the mid-term outcomes of Contegra implantation for the reconstruction of the right ventricular outflow tract to proximal branch pulmonary arteries in a multicentre study. METHODS: Between April 2013 and December 2019, 178 Contegra conduits were implanted at 5 Japanese institutes. The median age and body weight at operation were 16 months (25th-75th percentile: 8-32) and 8.3 kg (6.4-10.6). Sixteen patients were neonates (9.0%). Selected conduit sizes were 12 mm in 28 patients (15.7%), 14 mm in 67 patients (37.6%), 16 mm in 66 patients (37.1%), 18 mm in 5 patients (2.8%) and <12 mm in 12 patients (6.7%). Fifty-six grafts (31.4%) were ring supported. Proximal branch pulmonary arteries were concomitantly augmented in 85 patients (47.5%). Follow-up was completed in all patients and the median follow-up period was 3.1 years (1.3-5.1). RESULTS: The overall, conduit explantation-free and conduit infection-free survival rates at 5 years were 91.3%, 71.0% and 83.7%, respectively. Infection (P = 0.009) and common arterial trunk (P = 0.024) were risk factors for explantation. Conduit durability was shorter in smaller one (P < 0.001). Catheter interventions (for conduit to proximal branch pulmonary artery)-free survival rates at 5 years was 52.9%; however, need for catheter interventions was not a risk factor for conduit explantation. CONCLUSIONS: Mid-term outcomes of reconstruction of the right ventricular outflow tract to the proximal branch pulmonary arteries with Contegra were acceptable. The need for explantation over time was higher in smaller conduits. Conduit infection was a strong risk factor for conduit explantation. Frequently and repeated catheter interventions effectively extended the conduit durability.

The mid-term outcome of Fontan conversion compared with primary total cavopulmonary connection.

BACKGROUND: The indication of Fontan conversion (FC) from atriopulmonary connection (APC) to total cavopulmonary connection (TCPC) is unclear. We sought to analyze the mid-term outcome of prophylactic and therapeutic Fontan conversion compared with that of primary TCPC. METHODS: Patients with a univentricular heart who underwent cardiac catheterization at >18 years of age between July 2005 and July 2019 were included and divided into three groups: symptomatic APC patients who underwent therapeutic FC (t-FC, n = 13), asymptomatic APC patients after prophylactic FC (p-FC, n = 15), and patients who had primary TCPC procedure (pTCPC, n = 24). RESULTS: The mean last follow up was at the age of 32.0 ± 7.8, 26.8 ± 3.8, and 27.3 ± 7 years (p = 0.07) in t-FC, p-FC, and pTCPC, respectively. There was no late death. All of t-FC and 12 (80%) of p-FC cases underwent concomitant arrhythmic surgery. Consequently, five and four patients in t-FC and p-FC groups required pacemaker implantations mostly due to sinus node dysfunction. Thromboembolism was seen in 2 cases in both t-FC (15%) and p-FC (13%), and 1 case in pTCPC (4%) (p = 0.50). The last cardiac catheterization was performed at the age of 29.5 ± 8.5, 24.6 ± 3.8, and 26.3 ± 7.1 years (p = 0.11) in t-FC, p-FC, and pTCPC patients, respectively. There was no significant difference in central venous pressure, aortic pressure, and cardiac index among the three groups. There was no late supraventricular tachyarrhythmic event seen in t-FC and p-FC, whereas two patients in pTCPC had newly developed atrial flutter. CONCLUSIONS: FC is a safe and feasible procedure to bring APC patients back onto the same track of primary TCPC patients in terms of hemodynamics as well as arrhythmia. The antiarrhythmic procedure should be carefully chosen because sinus node dysfunction can frequently occur and FC itself would reduce the risk of arrhythmia.

[Comparative study between cryoablation and radiofrequency ablation for surgical intervention of atrial fibrillation associated with mitral valve disease].

OBJECTIVES: Recently, bipolar radiofrequency (RF) ablation has been introduced as a potential option to replace many of the Cox-maze incisions. We compared the surgical outcomes of maze procedures in patients who had undergone cryoablation and RF ablation and mitral valve surgery. METHODS: From January 2004 to August 2009, a total of 40 patients underwent the maze procedure and concomitant mitral operation as a 1st operation. Of these, the cryoablation maze procedure (n = 20) and RF ablation procedure (n = 20) were performed in the patients with similar background, although more patients in the RF group had undergone mitral valve repair (n = 15) compared to the cryoablation group (n = 8) [p = 0.025]. RESULTS: The peri-operative data, such as operative duration, cardiopulmonary bypass time, aortic cross-clamp time, postoperative ventilation time, and the duration of intensive care unit (ICU) stay did not show any significant difference between cryoablation group and RF group. Both ablation procedures were similarly effective in restoring sinus rhythm at the early post-operative period (cryoablation: 80%, RF: 70%). Nine patients in the cryoablation group and 13 patients in the RF ablation group required anti-arrhythmic medication. CONCLUSIONS: The RF ablation technique is simpler and equally effective in controlling atrial fibrillation compared to the cryoablation for the maze procedure of concomitant mitral valve surgery.

Dilated cardiomyopathy associated with elephant trunk in Loeys-Dietz syndrome.

A 14-year-old boy presented to our institution with a diagnosis of acute type A dissection. He was diagnosed with Loeys-Dietz syndrome and underwent aortic valve sparing repair at the age of 9 years. Emergency total arch repair with elephant trunk (ET) was performed successfully; echocardiogram before discharge showed normal left ventricular function and size. However, he was readmitted 1 month after discharge with significant left ventricular dysfunction and dilatation. The small folded ET caused a pressure gradient between the upper and lower body, which might deteriorate left ventricular function. Urgent balloon arterioplasty was performed to unfold the ET graft, resulting in no improvement of left ventricular function. ET removal and descending aorta replacement with an 18-mm graft was performed eventually. Left ventricular function and brain natriuretic peptide gradually improved after approximately 2 years of follow-up.

Management of partial anomalous pulmonary venous connection in single ventricle.

We herein report a case of a hypoplastic left heart syndrome variant complicated with partial anomalous pulmonary venous connection to the left innominate vein. We left the vertical vein at the time of the bidirectional Glenn procedure, and ligated the vertical vein at the time of the total cavopulmonary connection procedure, without reconnecting the vertical vein to the left atrium. Because of the development of an interlobar vein draining from the left upper lung into the lower lung after the bidirectional Glenn procedure, the circulation of the left upper lung was preserved after the total cavopulmonary connection procedure.

Expectations and limitations after bilateral pulmonary artery banding.

OBJECTIVES: Bilateral pulmonary artery banding (bil-PAB) has been developed as a part of a hybrid procedure for hypoplastic left heart syndrome (HLHS). We use this procedure for palliation of complex congenital heart disease, particularly in patients with arch anomaly. We reviewed our experience with bil-PAB. METHODS: Sixty-six consecutive cases between 2003 and 2014 were reviewed retrospectively. RESULTS: Fifty-one patients (77%) had single ventricle physiology (HLHS: 37, HLHS variant: 13, others: 1). Twelve patients had biventricular physiology [5 interrupted aortic arch with ventricular septal defect (VSD); 4 coarctation of the aorta with VSD; 2 truncus arteriosus; 1 other]. In 3 patients, it was difficult to determine if the physiology was single ventricle or biventricle due to borderline left ventricle size. Age and body weight at the time of operation were 4.3 ± 3.4 days and 2.7 ± 0.5 kg, respectively. Overall hospital mortality was 11% (7/66). Of the 42 patients from 2010, only 1 with multiple anomalies died between bil-PAB and the second-stage operation. Thirteen patients (20%) required reoperation of bil-PAB. The mean waiting time for the next operation was 44.3 ± 42.8 days. The body weight at the second-stage operation was 3.2 ± 0.8 kg. Seven (11%) patients developed patent ductus arteriosus obstruction during the waiting period. Twenty-four patients were 2.5 kg or less; 20 patients in this group progressed to the second-stage operation and of these 19 gained body weight. Forty-two patients were above 2.5 kg, but of these only 23 patients gained body weight. Ten patients underwent bil-PAB because of shock and 8 of these recovered from shock. In 2 borderline cases, the patients underwent the Norwood procedure and 1 patient had arch repair and VSD closure. CONCLUSIONS: Bil-PAB could be applied to a wide variety of complex diseases. Our mortality rates with bil-PAB improved significantly post-2010. Good indications for bil-PAB were shock and situations where it was unclear whether the physiology was single ventricle or biventricular. Body weight gain was difficult to predict, but patients weighing less than 2.5 kg could be expected to gain body weight after the procedure.

A successful patch angioplasty with auto-pulmonary wall for congenital coronary left main trunk occlusion in a young child.

Congenital occlusion of the left main coronary trunk is a life-threatening abnormality, and its optimal management remains controversial. This report describes a case of successful patch angioplasty with auto-pulmonary artery for a 12-year-old boy with congenital left main trunk occlusion. We divided the main pulmonary artery, harvested a pulmonary artery wall strip, and performed patch angioplasty of the occluded left main trunk ostium. We were able to clearly expose the left main trunk behind the pulmonary artery because the obstruction was divided for the patch material. The postoperative course was uneventful, and coronary angiography at 4 months after surgery showed excellent patency of the left main trunk. The auto-pulmonary arterial wall was easy to handle during angioplasty, and its favorable durability has been established both in the Ross procedures and in an arterial switch procedure. Therefore, we conclude that patch angioplasty using a piece of the pulmonary arterial wall represents a good alternative to conventional coronary artery bypass grafting.

Surgical repair of aortic regurgitation with left ventricular aneurysm diagnosed preoperatively as aortico-left ventricular tunnel.

Aortico-left ventricular tunnel (ALVT) is a rare congenital anomaly presenting abnormal connection between the ascending aorta and the left ventricle. In most reported cases, the aortic end of the tunnel is above the right coronary sinus. Cases of ALVT related to the left aortic sinus are extremely rare. We herein report a case diagnosed preoperatively as ALVT arising from the left aortic sinus. The actual diagnosis observed at surgery was aortic valve insufficiency with a left ventricular outflow tract aneurysm. We successfully performed aortic valve repair and plication of the left ventricular aneurysm.

Surgical outcome of Fontan conversion and arrhythmia surgery: Need a pacemaker?

BACKGROUND: Atrial tachyarrhythmias are frequent complications in the late period after the Fontan procedure, and important risk factors for a poor prognosis. The impact of Fontan conversion and arrhythmia surgery in failed Fontan patients has been described in many reports. OBJECTIVE: We evaluated our experience with Fontan conversion procedures, concomitant arrhythmia surgery, and pacemaker implantation. METHODS: We reviewed the hospital records of 25 consecutive patients who underwent a Fontan conversion procedure from January 2004 to March 2012. Twenty-four patients had arrhythmia surgery using cryoablation and radiofrequency ablation at the time of conversion. A bilateral atrial maze procedure was performed in 6 patients, right-side maze in 15, and isthmus block in 3. Three patients with a diagnosis of corrected transposition of the great arteries underwent simultaneous pacemaker implantation electively. RESULTS: There was no early death and one late death during a mean follow-up period of 21.2 months. Three tachyarrhythmia recurrences developed, and there were 4 occurrences of sinus bradycardia. Five of these patients required postoperative pacemaker implantation. CONCLUSION: The mid-term results of Fontan conversion and arrhythmia surgery in our institute were satisfactory. The occurrence of unexpected postoperative pacemaker requirement was high in the patients who underwent a right atrial or bilateral atrial maze procedure. Pacemaker or lead implantation is recommended for patients planned to undergo a right-side or full maze procedure.