Influence of the bronchus on the vascular growth of major aortopulmonary collateral arteries.

OBJECTIVES: Patients with major aortopulmonary collateral arteries (MAPCAs) often require additional surgical or catheter intervention after unifocalization (UF) due to stenosis and poor growth. We hypothesized that the UF design influences vascular growth; assessment was based on the passing route related to the bronchus. METHODS: We enrolled 5 patients with pulmonary atresia (PA), ventricular septal defect and MAPCA who underwent UF and subsequent definitive repair at our institute from 2008 to 2020. Angiography and computed tomography scans were routinely performed before surgical intervention to clarify pulmonary circulation and the relationships between MAPCAs and the bronchus, which revealed peculiar MAPCAs directed to the pulmonary hilum passing behind the bronchus (defined as retro-bronchial MAPCAs; rbMAPCAs). Vascular growth of rbMAPCAs, non-rbMAPCAs and the native pulmonary artery were assessed using the angiograms before and after repair. RESULTS: The angiogram before UF [age 42 (24-76) days, body weight 3.2 (2.7-4.2) kg] showed that the diameter of the original unilateral PA, rbMAPCA and non-rbMAPCA was 19.95 ± 6.65, 20.72 ± 5.36 and 20.29 ± 7.42 mm/m2, respectively (P = 0.917). UF was completed in a single-stage with the placement of modified Blalock-Taussig shunt through median sternotomy at the age of 1.6 (1.0-2.5) months. Angiograms performed 3.0 (1.0-10.0) years after UF completion demonstrated a smaller rbMAPCA diameter at peri-bronchial region (3.84 ± 2.84 mm/m2) compared to the native unilateral PAs (16.11 ± 5.46 mm/m2, P < 0.0001) and non-rbMAPCA (10.13 ± 4.44 mm/m2, P = 0.0103). CONCLUSIONS: RbMAPCAs tend to be stenosed at the point where they cross the bronchus and emerge in the middle mediastinum after in situ UF.

Risk analysis for patients with a functionally univentricular heart after systemic-to-pulmonary shunt placement.

OBJECTIVES: To investigate risk factors for mortality after systemic-to-pulmonary (SP) shunt procedures in patients with a functionally univentricular heart using the Japan Cardiovascular Surgery Database registry. METHODS: Clinical data from 75 domestic institutions were collected. Overall, 812 patients with a functionally univentricular heart who underwent initial SP shunt palliation were eligible for analysis. Patients with pulmonary atresia with an intact ventricular septum and patients with a SP shunt as part of the Norwood procedure were excluded. Risk factors for 30- and 90-day mortalities were analysed using a logistic regression model. RESULTS: Median age and body weight at SP shunt placement were 41 days and 3.6 kg, respectively. Modified Blalock-Taussig shunt, central shunt and other types of SP shunts were applied in 689 (84.9%), 94 (11.8%) and 30 (3.7%) patients, respectively. Cardiopulmonary bypass was utilized in 410 patients (51%) for 128 min (median, 19-561). There were 411 isolated SP shunt procedures. Median hospital stay was 27 days, and 742 (91.4%) patients were discharged. The 30- and 90-day mortality rates were 3.4% and 6.0%, respectively. Placement of a central shunt was identified as a risk factor for 30-day mortality, while lower body weight, preoperative ventilator support, right atrial isomerism and coexistence of major aortopulmonary collateral arteries and an unbalanced atrioventricular septal defect were identified as risk factors for 90-day mortality. CONCLUSIONS: SP shunt carries a high mortality rate in patients with a functionally univentricular heart when it is performed in smaller patients with complex cardiac anomalies.

[Valve-sparing Root Reimplantation for Stanford Type A Acute Aortic Dissection Combined with Aortic Root Dilation and Bicuspid Aortic Valve;Report of a Case].

A 45-year-old male developed Stanford type A acute aortic dissection combined with aortic root dilation and congenital bicuspid aortic valve (BAV). He had a Sieveres type 0 BAV, lateral subtype with right and left cusps. Valve-sparing root reimplantation was performed with decalcification of the cusps. Transthoracic echocardiography(TTE) at discharge revealed no aortic regurgitation, and peak velocity of BAV was 2.15 m/second, mean pressure gradient was 9.6 mmHg and aortic valve area was 2.15 cm2. TTE after 6 months revealed only slight elevation of the peak velocity to 2.78 m/second. To perform successful reimplantation in the case of BAV, anatomic orientation of the cusps should be approximately at 180° and the tissue of the cusps should either be normal or have only minor abnormalities. Valve-sparing root reimplantation for BAV needs a careful follow-up for progression of the aortic valve dysfunction.

Successful staged repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries in an extremely low birth weight infant.

An extremely low birth weight infant (810 g) was born with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries accompanied by pulmonary overcirculation, which eventually resulted in intestinal hypoperfusion and focal intestinal perforation from the very early stage of life. Based on the echographic findings, we performed banding operations twice to regulate the pulmonary blood flow on day 2 and day 9. At 6 months of age, a definitive repair simultaneous with unifocalization of major aortopulmonary collateral arteries was performed. At 1 year of age, the right ventricle/left ventricle pressure ratio was 0.44 after balloon angioplasty was performed for the right-sided pulmonary artery stenosis. The patient is in a stable condition and was followed-up for more than 2 years after definitive repair. This is the first known successful repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries in an extremely low birth weight infant.

Midterm results and risk factors of functional single ventricles with extracardiac total anomalous pulmonary venous connection.

OBJECTIVES: To evaluate the clinical outcomes of surgical repair of an extracardiac total anomalous pulmonary venous connection (TAPVC) in a functional single-ventricle (f-SV) strategy. METHODS: This was a retrospective analysis of 48 consecutive cases of extracardiac TAPVC repair, from 1998 to 2015. Demographic and clinical variables were as follows: median age, 24 (range 0-744) days; median weight, 3.1 (range 2.0-9.6) kg; type of TAPVC-supracardiac, 21 patients, infracardiac, 8, and mixed, 19; right atrial isomerism, 45 patients; pulmonary atresia, 24 patients; and obstructed TAPVC, 30 patients. Concomitant procedures included systemic-to-pulmonary shunting in 15 patients, pulmonary artery banding in 12, ventricle-to-pulmonary artery shunting in 2, a Norwood procedure in one, a bidirectional Glenn procedure in 16, and a Fontan procedure in 1. RESULTS: The 1- and 5-year cumulative survival rates were 66.0% and 58.0%, respectively. Of the 28 survivors, 22 (78.6%) underwent Fontan completion and 4 (14.3%) a bidirectional Glenn procedure, and 1 (3.6%) was awaiting a bidirectional Glenn procedure. Recurrent pulmonary venous stenosis (PVS) was observed in 17 patients, with a 1- and 5-year rates of freedom from recurrent PVS 59.8% and 53.5%, respectively. Of the 20 post-operative deaths, only 4 were PVS related. Only pre-operative pulmonary atresia was identified as an independent risk factor of mortality after TAPVC repair. CONCLUSIONS: The midterm surgical outcomes of f-SV with extracardiac TAPVC were acceptable. Moreover, among survivors, Fontan completion can be sufficiently expected. Further improvement, with the development of a comprehensive treatment strategy, is required for this patient group.

[Valve-sparing Replacement in Patients with Aortic Root Dilatation].

Valve-sparing root replacement is increasingly used to overcome drawbacks associated with valvular prostheses. In our institution, 7 patients underwent valve-sparing root replacement from August 2016 to July 2017. The mean age was 45 years (range, 14~69 years). Three patients had Marfan syndrome and 1 had Loeys-Dietz syndrome with acute aortic dissection. All patients underwent surgery with reimplantation technique using a Valsalva graft. Two patients required repair of aortic valve leaflet prolapse. All patients had an excellent clinical course, with mild or no aortic regurgitation and a decrease in end-diastolic volume on echocardiography. These results support the continued use of valve-sparing root replacement in selected patients.

One-stage definitive repair of complete atrioventricular septal defect and pulmonary atresia with major aortopulmonary collateral arteries.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is considered the most severe end of the tetralogy of Fallot spectrum, whereas complete atrioventricular septal defect associated with tetralogy of Fallot is a rare congenital defect. However, the combination of pulmonary atresia, major aortopulmonary collateral artery and complete atrioventricular septal defect is extremely rare, and surgical repair has not been reported. We describe a successful, 1-stage correction in a 7-month-old boy with this condition, who had an additional complication of a single aortic outlet from the right ventricle and significant atrioventricular valve regurgitation.

[Perioperative Care for Patients with Hypoplastic Left Heart Syndrome].

Hypoplastic Left Heart Syndrome (HLHS) is one of the most challenging congenital heart defects which require surgical interventions during neonatal period. In normal risk cohort, its surgical outcome has been improved dramatically since Dr. William Norwood reported the 1st successful case more than 30 years ago. The introduction of routine bilateral pulmonary artery banding with subsequent Norwood operation during early infantile( we call it as "rapid 2 stage Norwood operation") has brought an earlier hemodynamic stabilization after Norwood operation. However, the outcome of high risk patients, who are complicated with highly restrictive inter-atrial communication or who have significant tricuspid valve regurgitation from the early stage, is unsatisfactory even in current era. The 1st hospitalization with Norwood procedure is the beginning of staged palliations toward good Fontan circulation in the future. Very careful perioperative management is necessary to achieve survival discharge without complications.

Long-term outcome of patients with right atrial isomerism after common atrioventricular valve plasty.

OBJECTIVES: To review long-term outcomes of patients with right atrial (RA) isomerism who underwent common atrioventricular valve (CAVV) plasty. METHODS: We retrospectively analysed 59 patients with RA isomerism operated on between January 2004 and April 2016. We divided patients into those with CAVV plasty (CAVV plasty (+), 29 patients) and without CAVV plasty (CAVV plasty (-), 30), and we compared the outcome between the groups. We further divided patients into those with CAVV plasty before bidirectional cavopulmonary shunt (BCPS) operation (group before BCPS, 13 patients) or CAVV plasty with or after BCPS (group with or after BCPS, 16), and we compared the outcome between these groups. We reviewed the outcomes of 7 neonatal patients who underwent CAVV plasty. RESULTS: Kaplan-Meier estimated survival rates at 10 years were 70 ± 10% and 69 ± 9% in the CAVV plasty (+) and CAVV plasty (-) groups, respectively ( P = 0.45). Kaplan-Meier estimated survival rates at 10 years were 47 ± 17% and 85 ± 10% in the group before BCPS and group with or after BCPS, respectively ( P = 0.01). Among 7 neonates in the group before BCPS, 4 are alive; Kaplan-Meier estimated survival rates at 1 year and 5 years were 60 ± 20% and 30 ± 24%, respectively. CONCLUSIONS: Patients who underwent CAVV plasty with or after BCPS had good outcomes; the outcome of patients with CAVV plasty was the same as that of those without CAVV plasty. Treatment for patients who require CAVV plasty before BCPS, especially neonates, is challenging.

A successful staged Fontan operation for a right atrial isomerism neonate having major aortopulmonary collateral arteries and extracardiac total anomalous pulmonary venous connection.

Right atrial isomerism and pulmonary atresia combined with major aortopulmonary collateral arteries are very rare. Surgical treatment becomes more challenging when an extracardiac total anomalous pulmonary venous connection (TAPVC) requiring surgical repair neonatally is also present. We describe a successful staged Fontan operation for a neonate with a single ventricle, right atrial isomerism, pulmonary atresia with major aortopulmonary collateral arteries, a small central pulmonary artery and a supracardiac TAPVC. Unifocalization of the pulmonary vasculature was achieved by ligating all dual-supply major aortopulmonary collateral arteries at their origins, concomitant with supracardiac TAPVC repair and modified Blalock-Taussig shunt placement at 15 days. The patient underwent patch augmentation of the small stenosed central pulmonary artery at 8 months and bilateral bidirectional Glenn shunt at 14 months. The Fontan operation was completed at 27 months using an intra-extracardiac conduit with fenestration. One year later, catheter examination revealed good Fontan circulation with acceptable central venous pressure, oxygen saturation and cardiac function. We also describe a new angiographic concept of central pulmonary arteriography to visualize more accurately the complex pulmonary circulation system with both major aortopulmonary collateral arteries and a central pulmonary artery.

Modification of hemi-Fontan operation for patients with functional single ventricle and anomalous pulmonary venous connection to the superior vena cava: mid-term results.

OBJECTIVES: Fontan candidates with mixed totally anomalous pulmonary venous connection often have postoperative pulmonary venous obstruction after cavopulmonary anastomosis. Because some pulmonary venous obstructions have no intimal hypertrophy at reoperation, we considered such pulmonary venous obstructions to be caused by 3D deformities arising from dissection or mobilization of the vessels, and hypothesized that keeping the pulmonary venous branches in a natural position could avoid such obstruction. Here, we evaluated a modified hemi-Fontan strategy consisting of minimal dissection with no division of vessels and patch separation between systemic and pulmonary venous flow. METHODS: We retrospectively reviewed clinical records of infants with a functional single ventricle and supracardiac anomalous pulmonary venous connection who had undergone this procedure between 2002 and 2012. RESULTS: Nine infants underwent this procedure (median age, 5.6 months; range 3.2-30), all with right atrial isomerism and several pulmonary venous branches directly and separately connecting to the superior vena cava. In 5 patients, all pulmonary veins drained into the superior vena cava; in 1, the right pulmonary veins drained into the superior vena cava and in 3, a pulmonary venous branch drained into the superior vena cava. The median follow-up was 6.9 years (0.8-13 years). Three patients underwent reoperation for postoperative pulmonary venous obstruction caused by intimal hypertrophy; however, we confirmed no pulmonary venous obstruction caused by 3D deformities on the pulmonary venous branches connecting separately to the superior vena cava. Although 2 patients were effectively relieved from pulmonary venous obstruction, 1 died due to recurrent pulmonary venous obstruction. There was no late death and no sinus-node dysfunction. Eight patients underwent successful Fontan operation and catheterization. The median interval from the Fontan operation to the latest catheterization was 3.7 years (0.9-3.7 years). The median arterial oxygen saturation was 94% (91-97%) and the central venous pressure was 12 mmHg (8-14 mmHg); no deficiency of pulmonary arteries and veins was noted. CONCLUSIONS: For patients with functional single ventricle and anomalous pulmonary venous connections to the superior vena cava, our novel strategy of second-stage palliation could avoid postoperative pulmonary venous obstruction caused by 3D deformities, but may not eliminate pulmonary venous obstruction caused by intimal hypertrophy.

Efficacy of the 'intrapulmonary-artery septation' surgical approach for Fontan candidates with unilateral pulmonary arterial hypoplasia†.

OBJECTIVES: The bilaterally unbalanced development of pulmonary arteries (PAs), as a result of unilateral pulmonary arterial hypoplasia (PAhypo) makes patients either ineligible for the Fontan operation or candidates for the one-lung Fontan operation. In the present study, we examined the efficacy of intrapulmonary-artery septation (IPAS), a technique we reported in 2007 in which a septation is constructed within the central PA, in patients with unilateral PAhypo. METHODS: Sixteen patients with unilateral PAhypo and an affected PA index of ≤60 mm(2)/m(2), including non-confluent PA (NCPA), underwent IPAS between January 2000 and March 2012; patients with pulmonary venous obstruction were excluded from this study. We compared the affected PA index values before and after IPAS and after the Fontan operation as well as the bilateral pulmonary blood flow ratio using pulmonary scintigraphy. The post-Fontan operation values of central venous pressure (CVP), pulmonary vascular resistance (PVR), ventricular end-diastolic pressure (VEDP), cardiac index (CI) and arterial oxygen saturation (SaO2) at the most recent cardiac catheterization, as well as the pre-IPAS and post-Fontan New York Heart Association (NYHA) classification levels were examined. RESULTS: One patient died after IPAS (survival rate, 93.8%). Thirteen (86.7%) of the surviving 15 patients underwent the Fontan operation. The mean PA indexes were 25.5 ± 18.9 mm(2)/m(2) before IPAS and 67.9 ± 34.2 mm(2)/m(2) after IPAS (P = 0.003); the mean PA index was 71.1 ± 50.0 mm(2)/m(2) after the Fontan operation. Restenosis did not occur after the Fontan operation, and the PA diameters were maintained. The mean affected/unaffected lung blood flow ratio was 0.89 ± 0.67. The most recent cardiac catheterization after the Fontan operation indicated the following values: CVP, 11.7 ± 1.8 mmHg; PVR, 1.3 ± 0.4 U m(2); EDP, 5.7 ± 2.0 mmHg; CI, 3.1 ± 0.5 l/min/m(2) and SaO2, 94.9 ± 2.0%. The mean pre-IPAS and post-Fontan NYHA levels were 2.3 ± 0.6 and 1.2 ± 0.4, respectively (P = 0.0002). CONCLUSIONS: With IPAS, the affected PA diameters increased significantly, and were maintained after the Fontan operation, and continuity of the native PAs was achieved. IPAS is very effective for patients suffering from otherwise intractable diseases.

[Current Surgical Treatment for Patients with Hypoplastic Left Heart Syndrome, from the Viewpoint of Emergent Operation].

Hypoplastic left heart syndrome (HLHS) is one of the most challenging congenital heart defects which require surgical interventions during neonatal period. Since the 1st successful surgical repair was reported by Norwood et al. more than 30 years ago, some modifications in surgical maneuver [e. g. introduction of right ventricule (RV)-pulmonary artery (PA) shunt] and improvement of perioperative management have brought better outcome for this patient cohort. Recent years our institute have changed the treatment strategy from primary Norwood operation during neonatal period to bilateral PA banding and subsequent Norwood operation during early infantile (we call it as "rapid 2 stage Norwood operation"). Indeed, the introduction of this new strategy has lead to earlier hemodynamic stabilization after Norwood operation. Although these operations are performed electively for most of HLHS patients, emergent operations are necessary if they have restrictive inter-atrial communication with sever pulmonary congestion, or if their patent ductus arteriosus (PDA) s tend to close in spite of prostagrandine infusion, or if pulmonary over circulation develops so rapidly. Recently more than half of HLHS patients are diagonosed in fetal. The advances in fetal diagnosis allow us to find the patients earlier and to assess the severity of the disease. Most of HLHS patients who required emergent operation just after their birth are diagnosed in fetal. However, their surgical outcome is unsatisfactory so far. Treatment for these HLHS patients has become the next challenge.

Primary central pulmonary artery plasty for single ventricle with ductal-associated pulmonary artery coarctation.

BACKGROUND: Infants with a single ventricle who require a modified Blalock-Taussig shunt can have stenosis at the ductal insertion site on the pulmonary artery (PA). We hypothesized that primary PA plasty in the first palliation would limit PA stenosis and unbalanced pulmonary perfusion, and thereby facilitate safe accomplishment of the next operation. METHODS: From 1998 to 2012, functional single-ventricle patients with pulmonary atresia were managed as follows: initial operative strategy based on midline approach, using cardiopulmonary bypass, and central PA plasty (resection of all ductal tissue). All patients underwent catheter study and lung perfusion test before bidirectional cavopulmonary shunt (BCPS). RESULTS: Forty consecutive patients (heterotaxy, n = 27; others, n = 13) were enrolled in this study. Seven patients (17.5%) had repair of obstructed total anomalous pulmonary venous drainage, and 4 (10%) had repair of common atrioventricular valve at the first palliative operation. Fifteen patients (37.5%) underwent first palliation in the neonatal period. The mean PA index (mm(2)/m(2)) before BCPS was 230.7 ± 101.7. No pulmonary stenosis was detected before BCPS. Pulmonary artery diameter ratio (nonshunt side/shunt side) was 0.93 ± 0.25; pulmonary lung perfusion ratio (nonshunt side/shunt side) was 0.9 ± 0.2. A multivariate analysis identified persistent total anomalous pulmonary venous drainage (extracardiac type; p = 0.002) as a factor associated with unbalanced branch pulmonary arteries at BCPS. Thirty-five patients underwent BCPS, and 31 underwent Fontan procedures. There were 4 early deaths and 4 late deaths. CONCLUSIONS: The strategies we have employed facilitate safe accomplishment of the next-stage operation in diverse anatomic groups, with avoidance of unbalanced pulmonary vascular bed.

Mid- to long-term aortic valve-related outcomes after conventional repair for patients with interrupted aortic arch or coarctation of the aorta, combined with ventricular septal defect: the impact of bicuspid aortic valve†.

OBJECTIVES: Bicuspid aortic valve (BAV) is a common risk factor for valve-related problems and occurs more frequently in patients with an interrupted aortic arch (IAA) or coarctation of the aorta (CoA), combined with a ventricular septal defect (VSD), than in the general population. We have been using conventional repair for patients with IAA/CoA+VSD, including those with a very small aortic valve (AV). We retrospectively investigated the outcomes of these patients from the perspective of valve morphology. METHODS: Between 2000 and 2012, 50 consecutive patients underwent conventional repair for CoA/IAA with VSD [one-stage repair, 44 (88%); staged repair, 6 (12%)]. The criteria for conventional repair were as follows: an AV annulus diameter (AVD) z-score of >-6.0; mitral valve annulus diameter z-score of >-3.0; without retrograde flow in the proximal arch. Sixteen (32%) patients had BAV (Group B); the remaining 34 (68%) patients had a tricuspid AV (Group T). The surgical outcomes in both groups were investigated. RESULTS: No mortality occurred in the cohort. The median follow-up times were 6 years and 3 months (6 months to 11 years and 8 months) and 6 years and 2 months (4 months to 11 years and 4 months) in Groups B and T, respectively (P > 0.05). The preoperative data (median age at repair, median body weight and median AVD) were comparable in the two groups (P > 0.05). Two patients (4%) underwent reintervention in the aortic arch: 1 patient underwent balloon angioplasty for re-coarctation; the other removal of the interposed graft because of somatic growth. In both groups, the AVD became significantly larger at the 1-year follow-up, approximating the normal value. Three (6%) patients underwent a total of eight valve-related reinterventions (balloon angioplasty, 6; Ross operation, 1; valve replacement, 1). All three had BAV, and the AVD was 3.8-5.6 mm (z-score, -3.4 to -1.6). The 5-year valve-related reintervention-free survival rate was 76% and 100% in Groups B and T, respectively (P < 0.01). CONCLUSIONS: The long-term outcomes after conventional repair under our criteria were acceptable. BAV was a significant risk factor for valve-related reinterventions after conventional repair for IAA/CoA with VSD.

Modified Nikaidoh procedure with double-root translocation in a 1-year-old boy.

A 1-year-old boy weighing 10.4 kg underwent successful biventricular repair for transposition of the great arteries, a ventricular septal defect, and a left ventricular outflow tract (LVOT) obstruction with moderate pulmonary stenosis of the bicuspid pulmonary valve (z score of -4.4 for the pulmonary valve) by means of a modified Nikaidoh procedure with double root translocation by use of a valve-spared pulmonary root. The postoperative echocardiogram showed no LVOT obstruction, no aortic valve regurgitation, and mild pulmonary stenosis and pulmonary valve regurgitation. No reintervention has been required during the 6-year follow-up, with annular growth of the pulmonary valve.

Is routine rapid-staged bilateral pulmonary artery banding before stage 1 Norwood a viable strategy?

OBJECTIVE: We adopted a policy of rapid-staged bilateral pulmonary artery banding (bPAB) before the Norwood (NW) procedure for all patients with hypoplastic left heart syndrome. We hypothesized that this strategy might mitigate some of the traditional risk factors and that postponing a major bypass procedure beyond the newborn period could have both short- and long-term benefits. The purpose of the present study was to evaluate the efficacy of this strategy with respect to the short-term outcomes. METHODS: From 2008 to 2010, 14 patients underwent bPAB and maintenance of ductal patency with prostaglandin E1 infusion before stage 1 NW. For reference, we also reviewed the data from patients who had undergone the primary NW procedure in the 2 years immediately before the study period. RESULTS: The bPAB was performed at a median age of 6 days (range, 2-39), gestational age of 38.5 weeks (range, 36-41), and weight of 2.75 kg (range, 2.3-3.6). The subsequent NW was performed at a gestational age of 43.5 weeks (range, 41-51) and weight of 3.2 kg (range, 2.2-4.9). When the NW procedure was eventually performed on the pBAB group, the maximum blood lactate levels within the first 24 hours after the NW were lower than those in the earlier primary NW group (2.8±0.9 vs 10.1±6.5 mmol/dL, P=.0002) and the urine output in the first 24 hours after the NW was greater in the pPAB group (4.1±2.1 vs 2.2±1.5 mL/kg/h; P=.0051). CONCLUSIONS: These data suggest that rapid-staged bPAB before NW can reduce the challenge of postoperative management in the early postoperative period after the NW procedure and have potential to improve the outcomes.

Technical modification enabling pulmonary valve-sparing repair of a severely hypoplastic pulmonary annulus in patients with tetralogy of Fallot.

OBJECTIVES: Although pulmonary valve-sparing repair is preferable for patients with tetralogy of Fallot, the repair of very small pulmonary valves is challenging. The present study evaluates our modification for preserving severely hypoplastic pulmonary valves in patients with tetralogy of Fallot. METHODS: Sixty-eight consecutive patients who underwent complete repair of a tetralogy of Fallot between 2005 and 2011 were retrospectively reviewed. Patients with pulmonary atresia, absence of a pulmonary valve, atrioventricular septal defect and/or subarterial ventricular septal defect were excluded. There were 19 (28%) patients with a severely hypoplastic pulmonary annulus determined by preoperative echocardiography (z-score <-4). For these patients, we collected echocardiographic data and information about their postoperative course. RESULTS: Valve preserving was successful in 11 of 19 (58%) of the z < -4 group, compared with 48 of 49 (98%) of the z > -4 group. In the z < -4 valve-sparing subgroup (n = 11), the preoperative pulmonary valve diameter z-score was -4.9 (range -6.3 to -4.3), and an approach involving ventriculotomy with no transannular patch was employed at a mean age of 6.9 (range 2.2-16.1) months. In this subgroup, residual right ventricular outflow tract velocity was 2.4 ± 0.6 m/s at discharge from the hospital. During a mean follow-up of 2.6 ± 2.4 years, no reintervention was necessary. Late right ventricular outflow tract velocity was 2.2 ± 0.6 m/s, and there was no severe pulmonary regurgitation. The pulmonary valve annulus grew in relation to the patient's body surface area (z = -0.51, range -4.2-0.24) without any aneurysmal changes in the right ventricular outflow tract. CONCLUSIONS: Although our modification of valve-sparing repair for severely hypoplastic pulmonary valves in patients with tetralogy of Fallot could not be applied in all patients, this strategy enabled acceptable growth of the valve annulus, with only mild stenosis during the early to mid-term follow-up. This modification seems to be an option, even for a very small pulmonary valve.

Impact of postoperative hemodynamics in patients with functional single ventricle undergoing Fontan completion before weighing 10 kg.

BACKGROUND: Although the Fontan procedure is now being performed in younger patients, the influence of the early timing of Fontan on midterm to long-term results remains unclear. We investigated whether the timing of Fontan completion affects subsequent hemodynamics in patients with functional single ventricle followed for more than 3 years. METHODS: Between January 1997 and December 2008, 163 patients with functional single ventricle underwent extracardiac total cavopulmonary connection (TCPC) at a single institution. The survivors routinely underwent postoperative catheterization at 1 year and then every 5 years after TCPC and were divided into group A (weight < 10 kg; n = 65) and group B (weight > 10 kg; n = 97), and retrospectively reviewed. RESULTS: Mean follow-up was 6.6 ± 3.7 years. Mean weight and conduit size were 8.5 ± 0.8 kg and 17.1 ± 1.2 mm for group A versus 20.2 ± 13.1 kg and 18.8 ± 1.9 mm for group B, respectively (p < 0.001). There were 3 hospital deaths (group A, n = 1; group B, n = 2) and 5 late deaths (group A, n = 3; group B, n = 2). No TCPC was taken down. There were no thromboembolic events in either group. The end-diastolic volume of ventricle (eDV) (% of normal) was (232.7 ± 91.4 before TCPC versus 139.3 ± 57.2 5 years after TCPC; p < 0.001) in group A and (209.6 ± 77.7 before TCPC versus 136.7 ± 61.4 5 years after TCPC; p < 0.001) in group B. Ventricular ejection fraction and cardiac index at 5 years were similar in both groups. The end-diastolic pressure of ventricle (eDP) (mm Hg) at 1 year (p = 0.0037) and at 5 years (p = 0.047) was significantly lower in group A compared with group B. CONCLUSIONS: TCPC can be performed earlier with good intermediate results. Earlier unloading of a univentricular heart by means of TCPC might be advantageous for preservation of future ventricular function.