Is the QRS duration useful for determining the optimal timing of pulmonary valve replacement after tetralogy of Fallot repair?

OBJECTIVES: In pulmonary valve replacement (PVR) after tetralogy of Fallot (TOF) repair, the right ventricular end-diastolic and end-systolic volume index (RVEDVI and RVESVI) of cardiac magnetic resonance imaging (cMRI) are often used as indicators of the RV volume. We examined the utility of QRS duration, cardiothoracic ratio (CTR), and plasma brain natriuretic peptide (BNP) as indicators of the appropriate timing of cMRI to assess the RV volume and function before PVR. METHODS: We assessed the correlation of QRS duration, CTR, and BNP with RVEDVI and RVESVI on cMRI in 26 patients after TOF repair. Fifteen underwent PVR (age, 45.2 ± 11.4 years). Twelve underwent post-PVR cMRI. The RV volume change from before to after PVR was investigated. RESULTS: QRS duration, BNP, and CTR were positively correlated with RVEDVI and RVESVI after TOF repair. The post-PVR QRS duration was also positively correlated with post-PVR RVEDVI (p = 0.017) and RVESVI (p = 0.001). From before to after PVR, in 5 cases with QRS duration ≤ 160 ms, the QRS duration decreased from 110.4 ± 28.9 to 101.8 ± 30.5 ms (p = 0.063). Both RVEDVI and RVESVI decreased to the normal range in 4 of 5 cases. In contrast, in 7 cases with QRS duration > 160 ms, the QRS duration decreased from 183.0 ± 17.4 to 160.3 ± 23.8 ms (p = 0.013); however, RVESVI did not normalize in 6 of 7 cases. CONCLUSIONS: A prolonged QRS duration is a useful marker of RVEDVI and RVESVI enlargement after TOF repair. We recommend performing cMRI before the QRS duration reaches 160 ms due to normalization of the RV volume after PVR.

[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.

The efficacy and risk of cerebrospinal fluid drainage for thoracoabdominal aortic aneurysm repair: a retrospective observational comparison between drainage and non-drainage.

Objectives: We reviewed our experiences with thoracoabdominal aortic aneurysm (TAAA) repair to assess the efficacy of cerebrospinal fluid drainage (CSFD) to prevent the neurological deficits and complications associated with CSFD. Methods: Between 2002 and 2015, 118 patients underwent TAAA repair. Seventy-eight patients underwent CSFD for 2.7 ± 1.1 days after surgery. CSFD was not performed for the other 40 patients due to an urgent situation, chronic disseminated intravascular coagulation or anatomical difficulties. Results: There were 5 in-hospital deaths (4.2%). The neurological complications included paraplegia ( n = 14, 11.9%), paraparesis ( n = 3, 2.5%), cerebral infarction ( n = 11, 9.3%) and intracranial haemorrhage ( n = 1, 0.85%), none related to CSFD. The complications related to CSFD included headaches ( n = 13, 11.0%), subdural haematoma (which was treated conservatively) ( n = 1, 0.85%), a neurological symptom of the bilateral thighs ( n = 1, 0.85%), pale haemorrhagic discharge ( n = 2, 1.7%) and a fractured catheter ( n = 1, 0.85%). Eight patients had paraplegia and 1 patient had paraparesis among the 78 patients who underwent CSFD (9/78, 11.5%); among the 40 patients who did not undergo CSFD, 6 had paraplegia and 2 had paraparesis (8/40, 20.0%). A multivariate analysis demonstrated that CSFD had a significant protective effect for the spinal cord (odds ratio = 0.045, P = 0.007). Conclusions: CSFD effectively prevented spinal cord dysfunction in TAAA repair. However, some serious complications occurred, including subdural haematoma and a fractured catheter. It is therefore important to recognize both the efficacy and the risks of CSFD in TAAA repair.

Right ventricular centripetal plication: an aggressive right ventricular exclusion technique.

In patients with a functional single ventricle such as neonatal Ebstein's anomaly or pulmonary atresia with intact ventricular septum, the right ventricle can compress the left ventricle and decrease its performance due to the volume or pressure overload of the right ventricle. We have performed right ventricular centripetal plication from the inside to exclude the right ventricle and to minimize the adverse effect on the left ventricle and the results have been satisfactory.

Left Ventricular Outflow Tract Obstruction in Aortic Arch Anomalies With Ventricular Septal Defect.

BACKGROUND: The predictors of left ventricular outflow tract obstruction (LVOTO) after the repair of coarctation of the aorta or interruption of the aortic arch (CoA/IAA) with ventricular septal defect have been investigated. However, the predictors remain controversial. METHODS: We performed primary repair of CoA/IAA with ventricular septal defect for 75 patients from 1996 to 2005. Four of the 75 patients died within 5 years after primary repair without relation to LVOTO. The morphology of the aortic valve of 71 survivors was bicuspid in 23 patients and tricuspid in 48 patients. The mean follow-up was 9.2 ± 2.6 years after primary repair. RESULTS: There were 12 patients who showed LVOTO of 3.0 m/s or greater after primary repair. All of the 6 bicuspid patients demonstrated valvular aortic stenosis, and all of the 6 tricuspid patients showed discrete subvalvular LVOTO. In 5 of the 6 tricuspid patients, the aortic annular z-score before primary repair was -3.0 or less. A bicuspid aortic valve (p = 0.016) and the aortic annular z-score of -3.0 or less (p = 0.019) were significant risk factors for LVOTO after primary repair. At 10 years after primary repair, 82.6% and 95.6% of the bicuspid and tricuspid patients, respectively, were free from reoperation (p = 0.015). CONCLUSIONS: The presence of a bicuspid aortic valve and an aortic valve annular z-score of -3.0 or less before primary repair are risk factors for LVOTO, and stenotic bicuspid valves and discrete subvalvular LVOTO are the main causes of LVOTO after primary repair of CoA/IAA with ventricular septal defect. The bicuspid patients more frequently required reoperation than the tricuspid patients.

Expanding the Limits of Posterior Aortic Translocation: Biventricular Correction of Complex Transposition With Complete Atrioventricular Septal Defect and Heterotaxy.

This case report describes successful repair of d-transposition of the great arteries with severe left ventricular outflow tract obstruction and complete atrioventricular septal defect associated with heterotaxy by the use of posterior aortic translocation combined with repair of the atrioventricular septal defect and systemic venous anomalies.

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.

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.

Effects of tricuspid valve surgery on tricuspid regurgitation in patients with hypoplastic left heart syndrome: a non-randomized series comparing surgical and non-surgical cases.

OBJECTIVES: Tricuspid regurgitation (TR) remains a significant risk factor affecting the survival of patients with hypoplastic left heart syndrome (HLHS). We performed this study to investigate differences in the clinical course based on the timing of the development of TR and the effects of tricuspid valve surgery (TVS). METHODS: One hundred and five patients of classic HLHS underwent staged operations from May 1991 to July 2010. Forty-four patients (41.9%) exhibited moderate or greater TR during the follow-up. We defined the early TR group (30 patients, around the first palliative surgery) and the late TR group (14 patients, the later period) based on the timing of the appearance of moderate or greater TR. We performed TVS when moderate or greater TR was detected in 28 patients. The follow-up period was 5.5 ± 5.1 (plus/minus values are means ± SD) years (range: 0.01-14.6 years) after the first palliative surgery and 4.9 ± 4.4 years (range: 0.01-13.3 years) after TVS. RESULTS: The early TR group exhibited poorer survival than the late TR group (42.9 vs 92.9% at 5 years, P = 0.003). However, in the early TR group, the TVS significantly improved survival compared with that observed in the non-TVS cases (52.1 vs 23.3% at 5 years, P = 0.046). The right ventricular ejection fraction (RVEF) significantly decreased (62.7 ± 11.4 → 57.2 ± 12.6% (plus/minus values are means ± SD), P = 0.040) and the right ventricular end-diastolic diameter (RVDd) became significantly enlarged (27.7 ± 7.6 → 36.7 ± 3.4 mm, P < 0.001) in association with deterioration of the TR degree. TVS significantly improved the degree of TR (2.5 ± 0.5 → 1.5 ± 0.9°, P < 0.001) and RVDd (37.7 ± 7.4 → 30.4 ± 5.0 mm, P = 0.007); however, the RVEF was not improved 1 month after surgery (54.4 ± 12.1 → 54.3 ± 12.4%, P = 0.931) or at the latest follow-up (53.7 ± 14.9%, P = 0.836). CONCLUSIONS: The survival of HLHS patients who develop moderate or greater TR around the time of the first palliative surgery is worse than that of HLHS patients who develop moderate or greater TR at a later time. In this study, TVS for early TR improved survival and decreased right ventricular dimensions during the 4.9-year follow-up period.

[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.

Spontaneous disappearance of a saphenous vein graft pseudoaneurysm after coronary artery bypass grafting.

A saphenous vein graft pseudoaneurysm is a rare complication of coronary artery bypass grafting. Its natural course is largely unknown because there have been few observational studies of medically observed patients. We herein report a case of spontaneous regression of a saphenous vein graft pseudoaneurysm in the early postoperative period, which has never been described, to our knowledge, in the previous literature.

Twenty-eight years' experience of arterial switch operation for transposition of the great arteries in a single institution.

OBJECTIVES: We reviewed our 28 years of experience of arterial switch operation (ASO) for transposition of the great arteries to investigate late sequelae of this procedure. METHODS: 387 patients who underwent ASO from 1984 to 2010 were included in this retrospective study. The longitudinal data were estimated by the Kaplan-Meier method and compared using a log-rank test. Risk factors for late sequelae were analysed by the multivariable Cox proportional hazards model. RESULTS: The mean follow-up time was 10.0 years. There were 13 early deaths and 17 late deaths. All late deaths were within 1 year, except for three patients. Actuarial survival was 92.2 and 91.6% at 10 and 20 years, respectively. Sixty-six patients (17.1%) had developed pulmonary stenosis (PS) and 29 patients (7.5%) had developed moderate or more aortic insufficiency (AI) during follow-up. Selective coronary angiography was performed in 210 patients (54.3%) at 9.6 ± 5.1 years after ASO. Left main tract occlusion was found in 2 patients (2/210; 1.0%) and hypoplastic left coronary artery was found in 10 patients (10/210; 4.8%). Among these 12 patients, 8 patients were asymptomatic. Re-operation was performed in 76 patients (19.6%), pulmonary artery plasty for PS in 58 patients (15.0%), aortic valve replacement for AI including two Bentall operations in 9 patients (2.3%) and others. Freedom from re-operation was 78.2 and 62.8% at 10 and 20 years, respectively. The risk factor for PS was the use of equine pericardium for reconstruction (P < 0.0001). Factors associated with moderate or more AI was the presence of left ventricular outflow tract obstruction (P = 0.004). There were no risk factors for late coronary lesions. Three hundred and forty surviving patients (340/357; 95.2%) were in NYHA functional class I. Treadmill test, which was performed on 217 patients (56.1%) at 14.3 ± 5.4 years after ASO, revealed that the maximum heart rate was 97.5 ± 7.6% of normal and peak oxygen consumption was 105.2 ± 20.5% of normal. CONCLUSIONS: ASO was performed with satisfactory results in the overall survival and functional status. PS was the main reason for re-operation. Coronary lesions can appear late without any symptoms. Benefits of ASO can be achieved by long-term follow-ups of PS, AI and coronary lesions.

The impact of extracardiac conduit-total cavopulmonary connection on apicocaval juxtaposition.

OBJECTIVES: Modifications of the Fontan procedure are applied to a wide range of complex congenital heart defects with single ventricle physiology. We examined the pathway and the clinical results of extracardiac conduit-total cavopulmonary connection (EC-TCPC) for a malpositioned heart with apicocaval juxtaposition. METHODS: Of the 365 patients who underwent EC-TCPC since 1994, 56 patients with a malpositioned heart with apicocaval juxtaposition were included in this retrospective study (group 1). The pathway for the EC was selected after careful consideration of the results of preoperative angiography and computed tomography, as well as intra-operative findings. A concurrent group of 299 patients undergoing EC-TCPC without apicocaval juxtaposition was used as a control group (group 2). The mean follow-up periods for groups 1 and 2 were 5.5 years (range: 0-12 years) and 5.5 years (range: 0-14 years), respectively. RESULTS: The mean age at operation was 4.2±3.2 years and the median size of the conduit was 18 mm (range: 16-20mm). In 30 patients, the conduit was placed between the inferior vena cava (IVC) and the opposite side of the pulmonary artery crossing the vertebra. In another 25 patients, the conduit was positioned behind the ventricle between the IVC and on the same side as the pulmonary artery (PA). There was one patient who had a Y-style conduit placed between the IVC and right and left PAs behind the ventricle. In group 1, there were no early deaths; three patients died in the intermediate term due to gastric bleeding in one, haemoptysis in another and sudden death in yet another. None of the patients developed conduit stenosis or pulmonary venous obstruction, and no patient required re-operation. Three patients developed late complications, including arrhythmias requiring medication in two and subdural haematoma in one. The incidence of death or late complications did not differ among the pathways of conduits. In group 2, there were eight late deaths, and 27 patients developed late complications. The Kaplan-Meier survival rate was 93.5% at 5 and 10 years in group 1, and 97.3% at 5 years and 96.1% at 10 years in group 2 (log-rank test, P=0.29). The haemodynamics in groups 1 and 2 during the intermediate term were identical with respect to IVC pressure (8.9 ± 2.5 in group 1 and 9.6 ± 2.6mmHg in group 2), left ventricular end-diastolic pressure (4.4 and 4.3 mmHg, respectively), cardiac index (3.3 and 3.4 l min(-1)m(-2), respectively) and arterial oxygen saturation (94 and 94.2%, respectively). No patient in either group had a pressure gradient >2 mmHg between the IVC and central PA. Postoperative catheterisation data showed no significant differences in haemodynamics between the conduit pathways in group 1. CONCLUSIONS: EC-TCPC can be performed in children with apicocaval juxtaposition with excellent mid-term outcomes compared with other Fontan candidates. Based on individual cardiac anatomy, the pathway of the EC behind the ventricle or crossing the vertebra can be used without conduit stenosis or pulmonary venous obstruction.

[Repair of tetralogy of Fallot with obstruction of right pulmonary artery; report of a case].

Patients with tetralogy of Fallot showing unilateral obstruction of a pulmonary artery, especially the right pulmonary artery, are a high-risk group for pulmonary hypertension after repair. This case of tetralogy of Fallot with the obstruction of the right pulmonary artery received a Blalock-Taussig shunt at 7 months old, and the occluded right pulmonary artery caused empyema after surgery. At 2 years old, a cardiac catheter study showed a pulmonary artery index of 193.6 mm2/m2, so we undertook intracardiac repair. After the repair, she showed a relativery favorable clinical course. Systolic pulmonary artery pressure and right ventricular pressure were about 30 and 50 mmHg, respectively. We considered that tetralogy of Fallot with obstruction of right pulmonary artery could be repaired, as long as the pulmonary artery index was within the limits of indication and the left ventricle was well-developed.