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1.
Asian Cardiovasc Thorac Ann ; 32(2-3): 140-142, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38478425

ABSTRACT

A five-year-old boy was diagnosed with the ventricular septal rupture and ventricular aneurysm after blunt chest trauma in child abuse. Because of the intractable heart failure, he underwent operation in subacute period. Postoperative course was uneventful. The blunt cardiac injury in children can be caused by mild trauma and can be lethal. Surgical intervention should be considered when the clinical condition is unstable.


Subject(s)
Heart Aneurysm , Heart Failure , Thoracic Injuries , Ventricular Septal Rupture , Wounds, Nonpenetrating , Child, Preschool , Humans , Male , Heart Aneurysm/diagnostic imaging , Heart Aneurysm/etiology , Heart Aneurysm/surgery , Heart Failure/etiology , Thoracic Injuries/complications , Thoracic Injuries/diagnostic imaging , Ventricular Septal Rupture/diagnostic imaging , Ventricular Septal Rupture/etiology , Ventricular Septal Rupture/surgery , Wounds, Nonpenetrating/complications , Wounds, Nonpenetrating/diagnostic imaging
2.
Article in English | MEDLINE | ID: mdl-37095746

ABSTRACT

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.

3.
Eur J Cardiothorac Surg ; 60(2): 377-383, 2021 07 30.
Article in English | MEDLINE | ID: mdl-33712829

ABSTRACT

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.


Subject(s)
Blalock-Taussig Procedure , Heart Defects, Congenital , Univentricular Heart , Heart Defects, Congenital/surgery , Humans , Infant , Palliative Care , Pulmonary Artery/surgery , Retrospective Studies , Risk Assessment , Treatment Outcome
4.
Surg Case Rep ; 6(1): 280, 2020 Nov 05.
Article in English | MEDLINE | ID: mdl-33151421

ABSTRACT

BACKGROUND: Staphylococcus lugdunensis is a coagulase-negative Staphylococcus species, which are weak pathogenic bacteria generally. However, the acute and severe pathogenicity of Staphylococcus lugdunensis infective endocarditis may be due to the rapid growth of large vegetation and consequent valve destruction. CASE PRESENTATION: The patient was an 81-year-old male who visited our hospital with chief complaints of low back pain and high fever. Four years before this visit, he had undergone aortic valve replacement for aortic regurgitation. He was found to be hypotensive. Although there is no heart murmur on auscultation and echocardiography revealed negative findings with aortic valve, a blood test showed increases in the white blood cell count and C-reactive protein concentration. On the next day, Gram-positive cocci were detected in a blood culture and echocardiography detected a large vegetation on the prosthetic valve with increased flow velocity. Therefore, he underwent redo aortic valve replacement emergently. Staphylococcus lugdunensis was identified in blood samples and vegetation culture. Consequently, the patient was treated with antibiotics for 5 weeks after the operation and discharged home. CONCLUSIONS: We experienced rapidly progressive prosthetic valve endocarditis caused by Staphylococcus lugdunensis. Hence, Staphylococcus lugdunensis infective endocarditis requires aggressive treatment, and the pathogenicity of this coagulase-negative Staphylococcus with high drug susceptibility should not be underestimated.

5.
Kyobu Geka ; 73(11): 936-939, 2020 Oct.
Article in Japanese | MEDLINE | ID: mdl-33130718

ABSTRACT

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.


Subject(s)
Aortic Dissection , Aortic Valve , Aortic Dissection/complications , Aortic Dissection/diagnostic imaging , Aortic Dissection/surgery , Aortic Valve/abnormalities , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Bicuspid Aortic Valve Disease , Dilatation , Heart Valve Diseases , Humans , Male , Middle Aged , Replantation , Retrospective Studies
6.
Gen Thorac Cardiovasc Surg ; 68(6): 637-640, 2020 Jun.
Article in English | MEDLINE | ID: mdl-31147961

ABSTRACT

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.


Subject(s)
Abnormalities, Multiple/surgery , Aorta/surgery , Heart Septal Defects, Ventricular/surgery , Pulmonary Artery/surgery , Pulmonary Atresia/surgery , Angioplasty, Balloon , Aorta/abnormalities , Collateral Circulation , Female , Heart Septal Defects, Ventricular/complications , Humans , Infant , Infant, Extremely Low Birth Weight , Infant, Newborn , Lung/blood supply , Pulmonary Artery/abnormalities , Pulmonary Atresia/complications , Stenosis, Pulmonary Artery/surgery
7.
Gen Thorac Cardiovasc Surg ; 67(11): 941-948, 2019 Nov.
Article in English | MEDLINE | ID: mdl-31134530

ABSTRACT

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.


Subject(s)
Cardiovascular Surgical Procedures/methods , Heterotaxy Syndrome/surgery , Pulmonary Atresia/surgery , Pulmonary Veins/surgery , Scimitar Syndrome/surgery , Child, Preschool , Female , Fontan Procedure , Humans , Infant , Infant, Newborn , Male , Norwood Procedures , Pulmonary Artery/abnormalities , Pulmonary Artery/surgery , Pulmonary Veins/abnormalities , Recurrence , Retrospective Studies , Risk Factors , Stenosis, Pulmonary Vein/etiology , Survival Rate , Time Factors , Univentricular Heart/surgery
9.
Kyobu Geka ; 71(1): 19-24, 2018 Jan.
Article in Japanese | MEDLINE | ID: mdl-29483476

ABSTRACT

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.


Subject(s)
Aortic Valve Insufficiency/diagnostic imaging , Aortic Valve Insufficiency/surgery , Blood Vessel Prosthesis Implantation , Adolescent , Adult , Aged , Female , Humans , Imaging, Three-Dimensional , Male , Middle Aged , Tomography, X-Ray Computed , Young Adult
10.
Interact Cardiovasc Thorac Surg ; 26(2): 343-345, 2018 02 01.
Article in English | MEDLINE | ID: mdl-29049668

ABSTRACT

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.


Subject(s)
Abnormalities, Multiple , Aorta, Thoracic/surgery , Cardiac Surgical Procedures/methods , Collateral Circulation , Heart Septal Defects/surgery , Pulmonary Artery/surgery , Pulmonary Atresia/surgery , Anastomosis, Surgical/methods , Angiography , Aorta, Thoracic/diagnostic imaging , Echocardiography , Female , Follow-Up Studies , Heart Septal Defects/diagnosis , Humans , Imaging, Three-Dimensional , Infant , Male , Pulmonary Artery/diagnostic imaging , Pulmonary Atresia/diagnosis , Time Factors
11.
Kyobu Geka ; 70(8): 622-626, 2017 07.
Article in Japanese | MEDLINE | ID: mdl-28790278

ABSTRACT

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.


Subject(s)
Hypoplastic Left Heart Syndrome/surgery , Norwood Procedures , Perioperative Care/methods , Heart Septal Defects, Atrial/complications , Humans , Infant , Retrospective Studies , Risk Factors , Treatment Outcome , Tricuspid Valve Insufficiency/complications
12.
Eur J Cardiothorac Surg ; 51(5): 987-994, 2017 May 01.
Article in English | MEDLINE | ID: mdl-28329375

ABSTRACT

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.


Subject(s)
Heart Valves/surgery , Heterotaxy Syndrome/mortality , Heterotaxy Syndrome/surgery , Blalock-Taussig Procedure , Child, Preschool , Female , Fontan Procedure , Heart Valve Diseases/surgery , Heterotaxy Syndrome/epidemiology , Humans , Infant , Infant, Newborn , Kaplan-Meier Estimate , Male , Retrospective Studies , Treatment Outcome
13.
Interact Cardiovasc Thorac Surg ; 24(1): 135-137, 2017 01.
Article in English | MEDLINE | ID: mdl-27628651

ABSTRACT

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.


Subject(s)
Abnormalities, Multiple , Aorta, Thoracic/surgery , Fontan Procedure/methods , Heart Ventricles/surgery , Heterotaxy Syndrome/surgery , Pulmonary Veins/surgery , Scimitar Syndrome/surgery , Angiography , Aorta, Thoracic/abnormalities , Aorta, Thoracic/diagnostic imaging , Blalock-Taussig Procedure/methods , Echocardiography , Heterotaxy Syndrome/diagnosis , Humans , Infant, Newborn , Male , Pulmonary Veins/abnormalities , Scimitar Syndrome/diagnosis
14.
Interact Cardiovasc Thorac Surg ; 22(5): 546-52, 2016 05.
Article in English | MEDLINE | ID: mdl-26860898

ABSTRACT

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.


Subject(s)
Abnormalities, Multiple , Fontan Procedure/methods , Heart Defects, Congenital/surgery , Heart Ventricles/surgery , Pulmonary Veins/abnormalities , Pulmonary Veno-Occlusive Disease/surgery , Vascular Malformations/surgery , Child, Preschool , Female , Follow-Up Studies , Heart Ventricles/abnormalities , Humans , Infant , Male , Pulmonary Veins/surgery , Pulmonary Veno-Occlusive Disease/etiology , Retrospective Studies , Time Factors
15.
Eur J Cardiothorac Surg ; 49(1): 183-7, 2016 Jan.
Article in English | MEDLINE | ID: mdl-25769466

ABSTRACT

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.


Subject(s)
Fontan Procedure , Pulmonary Artery/abnormalities , Pulmonary Artery/surgery , Vascular Malformations/surgery , Adolescent , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Male , Retrospective Studies , Treatment Outcome , Vascular Malformations/mortality , Young Adult
16.
Kyobu Geka ; 68(8): 629-33, 2015 Jul.
Article in Japanese | MEDLINE | ID: mdl-26197906

ABSTRACT

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.


Subject(s)
Hypoplastic Left Heart Syndrome/surgery , Cardiac Surgical Procedures , Humans , Hypoplastic Left Heart Syndrome/diagnosis , Prenatal Diagnosis , Risk Factors , Treatment Outcome
17.
Ann Thorac Surg ; 98(3): 919-26, 2014 Sep.
Article in English | MEDLINE | ID: mdl-25038976

ABSTRACT

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.


Subject(s)
Heart Defects, Congenital/surgery , Heart Ventricles/abnormalities , Heart Ventricles/surgery , Pulmonary Artery/surgery , Cardiac Surgical Procedures/methods , Feasibility Studies , Female , Heart Defects, Congenital/complications , Humans , Infant , Infant, Newborn , Male , Retrospective Studies , Vascular Surgical Procedures/methods
18.
Eur J Cardiothorac Surg ; 46(6): 952-60; discussion 960, 2014 Dec.
Article in English | MEDLINE | ID: mdl-24616392

ABSTRACT

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.


Subject(s)
Aorta, Thoracic/surgery , Aortic Coarctation/surgery , Aortic Valve/abnormalities , Heart Septal Defects, Ventricular/surgery , Heart Valve Diseases/surgery , Aortic Coarctation/complications , Aortic Valve/surgery , Bicuspid Aortic Valve Disease , Child , Child, Preschool , Heart Septal Defects, Ventricular/complications , Heart Valve Diseases/complications , Humans , Infant , Infant, Newborn , Postoperative Complications/etiology , Retrospective Studies , Treatment Outcome
19.
Ann Thorac Surg ; 97(3): 1055-7, 2014 Mar.
Article in English | MEDLINE | ID: mdl-24580922

ABSTRACT

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.


Subject(s)
Abnormalities, Multiple/surgery , Aortic Valve/surgery , Heart Septal Defects, Ventricular/surgery , Pulmonary Valve Stenosis/surgery , Pulmonary Valve/surgery , Transposition of Great Vessels/surgery , Ventricular Outflow Obstruction/surgery , Cardiac Surgical Procedures/methods , Heart Septal Defects, Ventricular/complications , Humans , Infant , Male , Pulmonary Valve Stenosis/complications , Transposition of Great Vessels/complications , Ventricular Outflow Obstruction/complications
20.
J Thorac Cardiovasc Surg ; 148(4): 1519-25, 2014 Oct.
Article in English | MEDLINE | ID: mdl-24472315

ABSTRACT

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.


Subject(s)
Hypoplastic Left Heart Syndrome/surgery , Norwood Procedures , Pulmonary Artery/surgery , Alprostadil/therapeutic use , Female , Humans , Infant , Infant, Newborn , Male , Retrospective Studies , Risk Factors , Survival Rate , Treatment Outcome
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