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Congenital heart disease (CHD) is common among patients with trisomy 18 (T18), but cardiac surgery has been rarely indicated for T18 patients due to their short life span. Although the therapeutic effects of aggressive interventions were recently demonstrated for T18 patients, the subjects and factors examined varied, resulting in inconsistent findings. Therefore, the effects of cardiac surgery for T18 remain unclear. We herein investigated the outcomes of cardiac palliative surgery for CHD with increased pulmonary blood flow in T18 patients. 27 patients were examined: 13 (48.1%) underwent cardiac palliative surgery and 14 (51.9%) did not. Median survival times in the no-surgery and surgery groups were 223.0 days (95% confidence interval [CI]: 46-361 days) and 723.0 days (95% CI: 360-1447 days), respectively. The number of patients with pulmonary hypertension significantly differed between the two groups (5 of 14 in the no-surgery group and 0 in the surgery group). Five of 14 patients in the no-surgery group and 10 of 13 in the surgery group were discharged to home care (odds ratio: 10.8 [95% CI: 1.07-110.0]). Therefore, cardiac palliative surgery may be used to treat CHD with increased pulmonary blood flow in T18 patients.
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The "Avalon Elite®" cannula is a double-lumen cannula used to establish veno-venous extracorporeal membrane oxygenation support. The reported advantages are that extracorporeal circulation can be established by cannulating through the right internal jugular vein only, and there is less re-circulation than with a two-cannula technique. It is available in a wide range of cannula sizes and can be used in a variety of patients, from children to adults. We herein report three pediatric cases in which an Avalon Elite® cannula was useful. The first was a case of acute mitral regurgitation due to idiopathic chordal rupture for postoperative severe lung injury and atelectasis due to cardiogenic pulmonary edema. The second was a case of end-stage radiation pneumonitis for safe transfer to facility of lung transplantation. The third was a convalescent case of fulminant myocarditis with severe atelectasis due to cardiogenic pulmonary edema. In each case, veno-venous extracorporeal membrane oxygenation using an Avalon Elite® cannula was established, the expected sufficient support was secured, and a good clinical course was obtained without major complications associated with an Avalon Elite® cannula.
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In the Fontan circulation, there is a substantial degree of systemic-to-pulmonary collateral flow (SPCF), which can be measured by cardiac magnetic resonance (CMR). However, the correlation between the degree of SPCF and long-term outcomes is not fully understood. We retrospectively studied 321 patients who underwent the Fontan procedure and CMR at a single center. Using CMR, we calculated SPCF as pulmonary blood flow - systemic blood flow. %SPCF was defined as SPCF ÷ pulmonary blood flow. The mean age of patients at CMR was 14.3 ± 7.5 years. The average %SPCF was 13.0% ± 11.0%. With a multivariate analysis, %SPCF was significantly correlated with time (i.e., the longer the time period since the Fontan procedure, the lower the %SPCF) (p = 0.006), previous total anomalous pulmonary vein drainage (p = 0.007), a low pulmonary artery index (Nakata index) before the Fontan procedure (p = 0.04), and older age at the time of the Fontan procedure (p = 0.002). Regarding the findings after the Fontan procedure, %SPCF was significantly correlated with ventricular end-diastolic volume (p < 0.001), ventricular end-systolic volume (p < 0.001), central venous pressure (p < 0.001), plasma brain natriuretic peptide concentration (p < 0.001), hemoptysis (p = 0.009), and poor New York Heart Association functional class (p = 0.007). SPCF was correlated with clinical condition after the Fontan procedure. The importance of sufficient growth of the pulmonary vascular bed should be emphasized because the development of SPCF is believed to result from the poor condition of the pulmonary circulation.
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Procedimiento de Fontan/métodos , Cardiopatías Congénitas/cirugía , Circulación Pulmonar , Adolescente , Velocidad del Flujo Sanguíneo , Niño , Femenino , Cardiopatías Congénitas/fisiopatología , Ventrículos Cardíacos/fisiopatología , Hemoptisis , Humanos , Imagen por Resonancia Magnética , Masculino , Análisis Multivariante , Arteria Pulmonar/fisiopatología , Estudios Retrospectivos , Volumen Sistólico , Adulto JovenRESUMEN
Various pathologies of heterotaxy should be diagnosed by documenting the arrangement of the cardiac structures and other organs. They carry functional derangements of cardiovascular systems and abdominal organs, such as atrioventricular valve dysfunction, pulmonary vein obstruction, arrhythmia, and intestinal malrotation. An appropriately organized management of these malformations is pivotal for improving outcomes.
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Síndrome de Heterotaxia , Vólvulo Intestinal , Corazón Univentricular , Arritmias Cardíacas , Síndrome de Heterotaxia/diagnóstico por imagen , Síndrome de Heterotaxia/cirugía , HumanosRESUMEN
PURPOSE: The aim of this study was to investigate the resolution property in XY-plane for half-reconstruction computed tomography (CT) image by measuring 360° multi-directional modulation transfer functions (MTFs). MATERIALS AND METHODS: The 360° multi-directional MTFs were measured by use of a wire method to obtain line spread function with 15° interval in XY-plane. The MTFs of half-reconstruction CT image were measured with 100 mm off-center positions on the X- and Y-axis (X+100 mm, X-100 mm, Y+100 mm, and Y-100 mm) and compared with those of full-reconstruction CT image. We measured the MTFs of the half-reconstruction CT image at X+100 mm position with various X-ray tube positions of projection dataset. RESULTS: There were obvious differences for the MTFs of the half-reconstruction CT image between the tangential and radial directions at each measurement position. The dependences of the resolution property for the half-reconstruction CT image on positions and directions in XYplane were similar to those for the full-reconstruction CT image. The higher and the lower MTFs of the half-reconstruction CT image at X+100 mm position were measured with X-ray tube position of projection dataset at +X side and at -X side compared with those of the full-reconstruction CT image, respectively. CONCLUSION: We conclude that the half-reconstruction CT image had similar resolution property in XY-plane to the full-reconstruction CT image and showed dependency on the X-ray tube position of projection dataset for MTF in the tangential direction.
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Algoritmos , Tomografía Computarizada por Rayos X , Fantasmas de ImagenRESUMEN
BackgroundsThe aim of this study was to assess the impact of age at bidirectional cavopulmonary anastomosis on haemodynamics after total cavopulmonary connection. METHODS: We conducted a retrospective analysis of 100 consecutive patients who underwent total cavopulmonary connection from 2010 to 2014. All patients had previously undergone bidirectional cavopulmonary anastomosis. These patients were classified into two groups according to age at bidirectional cavopulmonary anastomosis: younger group, 6 months (n=67). RESULTS: The proportion of hypoplastic left heart syndrome was higher in the younger group (48 versus 4%). After total cavopulmonary connection, the chest tube period was longer in the younger group (10.1±6.6 versus 6.7±4.5 days; p=0.009). Catheterisation 6 months after total cavopulmonary connection revealed that pulmonary artery pressure was higher (11.5±1.9 versus 10.4±2.1 mmHg; p=0.017) and Nakata index was lower (219±79 versus 256±70 mm2/m2; p=0.024) in the younger group. In patients with a non-hypoplastic left heart syndrome, there was no difference in post-operative haemodynamics between two groups, but the total amount of chest drainage after total cavopulmonary connection was larger in the younger group (109±95 versus 55±40 ml/kg; p=0.044). CONCLUSIONS: Early bidirectional cavopulmonary anastomosis did not affect the outcome of total cavopulmonary connection. Longer chest tube period, smaller pulmonary artery, and higher pulmonary artery pressure after total cavopulmonary connection were recognised in early bidirectional cavopulmonary anastomosis patients, especially in hypoplastic left heart syndrome.
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Puente Cardíaco Derecho/métodos , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Arteria Pulmonar/fisiopatología , Factores de Edad , Femenino , Procedimiento de Fontan , Puente Cardíaco Izquierdo , Hemodinámica , Hospitales Pediátricos , Humanos , Lactante , Japón , Masculino , Análisis de Regresión , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
A 4-month-old girl with Down syndrome showed unexpected deterioration of pulmonary hypertension. Despite aggressive pulmonary vasodilation therapy, the patient died at 5 months of age. Lung autopsy showed that the pulmonary veins were obliterated by intimal fibrous thickening, and the media of the veins was arterialised with an increase in elastic fibres. Pulmonary veno-occlusive disease should be considered in the management of individuals with Down syndrome.
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Síndrome de Down/complicaciones , Síndrome de Down/patología , Hipertensión Pulmonar/etiología , Enfermedad Veno-Oclusiva Pulmonar/diagnóstico , Enfermedad Veno-Oclusiva Pulmonar/patología , Autopsia , Cateterismo Cardíaco , Resultado Fatal , Femenino , Humanos , Hipertensión Pulmonar/tratamiento farmacológico , Lactante , Pulmón/patología , Venas Pulmonares/patologíaRESUMEN
The purpose of this study was to investigate the effect of the position in XYZ directions and acquisition parameters on the basic image qualities of for cone beam computed tomography (CBCT) in an angiography system with flat panel detector. The resolution property (modulation transfer function: MTF) and the noise property (Wiener spectrum: WS) of CBCT images in X-Y plane were measured with different acquisition parameters (scan matrix number and projection number) and the effect of the position in XYZ directions. The MTFs with 1024×1024 matrix were higher than those of 512×512 matrix and decreased in the peripheral areas due to the reduction of projection number. The highest and the lowest MTFs were measured at the X-ray tube side and on the detector side of the position in X-Y plane, respectively. The WS-doubled projection number showed about 50% lesser noise level. There were differences in the Wiener spectra (WS) at the position in XYZ directions. We conclude that the resolution and the noise property of CBCT image in X-Y plane showed dependences on the position in XYZ directions and acquisition parameters of the CBCT.
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Angiografía/instrumentación , Angiografía/métodos , Tomografía Computarizada de Haz Cónico/instrumentación , Tomografía Computarizada de Haz Cónico/métodos , Aumento de la Imagen/métodos , HumanosRESUMEN
OBJECTIVES: After staged reconstruction for hypoplastic left heart syndrome, the neoaortic root tends to dilate, and the incidence of significant neoaortic valve insufficiency increases with time. This study aimed to evaluate the mid-term outcomes of the neoaortic root geometries and valve function after chimney reconstruction in the Norwood procedure. METHODS: Between 2013 and 2021, 20 consecutive patients who underwent chimney reconstruction during the Norwood procedure for hypoplastic left heart syndrome and its variants in our institution were enrolled. The actual diameters of the following points were measured, and Z-scores were calculated based on the normal aortic root geometries using the long axis view of echocardiography at the pre-Norwood stage and the lateral view of angiography at pre-Glenn, pre-Fontan, post-Fontan and follow-up (age 5-6 years) stages: neoaortic valve annulus; sinus of Valsalva; sinotubular junction; and ascending aorta just proximal to the anastomosis to the aortic arch. The degree of neoaortic valve regurgitation was evaluated by echocardiography at each stage. RESULTS: The median follow-up period was 3.9 years. Neoaortic roots after chimney reconstruction were spared from progressive dilation over time. With growth, the conical configuration of the neoaortic roots was preserved without geometrical distortion. The Z-scores of the annulus, sinus of Valsalva, sinotubular junction and ascending aorta ranged roughly from 4 to 6, 4 to 6, 2 to 4 and 0 to 2, respectively. All neoaortic valves at each stage had mild or no regurgitation. CONCLUSIONS: Chimney reconstruction prevented neoaortic root dilation and avoided significant neoaortic valve regurgitation in the mid-term. These neoaortic dimensions with smooth flow profiles in the neoaorta after chimney reconstruction may have contributed to the current results. Further studies are needed to clarify the long-term outcomes.
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Insuficiencia de la Válvula Aórtica , Síndrome del Corazón Izquierdo Hipoplásico , Procedimientos de Norwood , Humanos , Preescolar , Niño , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Resultado del Tratamiento , Procedimientos de Norwood/efectos adversos , Aorta/cirugía , Ecocardiografía , Insuficiencia de la Válvula Aórtica/cirugía , Insuficiencia de la Válvula Aórtica/etiología , Estudios de Seguimiento , Estudios RetrospectivosRESUMEN
Aortic regurgitation in association with aortic stenosis is rare in the fetus. Findings have shown that severe aortic regurgitation is worsened by the increase in systemic vascular resistance after birth, resulting in low cardiac output, hypoxemia, and neonatal death. This report describes a unique case of aortic regurgitation with aortic stenosis, severe mitral regurgitation, retrograde flow in the aortic arch, and an enormous left atrium with a restrictive foramen ovale in a fetus. In this case, aortic regurgitation was diminished immediately after birth, indicating that spontaneous improvement in aortic regurgitation after birth should be taken into account when the final prognosis is predicted.
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Insuficiencia de la Válvula Aórtica/diagnóstico por imagen , Estenosis de la Válvula Aórtica/diagnóstico por imagen , Foramen Oval Permeable/diagnóstico por imagen , Insuficiencia de la Válvula Mitral/diagnóstico por imagen , Adulto , Insuficiencia de la Válvula Aórtica/cirugía , Estenosis de la Válvula Aórtica/cirugía , Gasto Cardíaco Bajo , Ecocardiografía , Femenino , Procedimiento de Fontan , Foramen Oval Permeable/cirugía , Atrios Cardíacos/anomalías , Humanos , Insuficiencia de la Válvula Mitral/cirugía , Procedimientos de Norwood , Embarazo , Respiración Artificial , Ultrasonografía PrenatalRESUMEN
For the purpose of examining the clinical applicability of a newly developed surgical sealant, animal experiments were performed, and clinical trial was followed. In animal experiments, several animal models, including carotid artery anastomosis model and coronary artery bypass grafting model were undertaken. In each model, complete hemostasis of the anastomoses using four simple interrupted sutures, was obtained. In addition, elastomeric property of the sealant prevented thinning of the arterial wall. The clinical trial performed in patients with thoracic aortic surgery showed significantly better hemostasis even under heparinized condition. Based on these excellent results, clinical usage of the sealant was approved.
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Arterias/cirugía , Técnicas Hemostáticas/instrumentación , Adhesivos Tisulares/uso terapéutico , Animales , Aneurisma de la Aorta Torácica/cirugía , Perros , HumanosRESUMEN
We report the case of a 19-month-old girl with a right-dominant unbalanced atrioventricular septal defect and severe right-sided atrioventricular valve regurgitation who underwent biventricular repair using basal chordae resection, artificial chordae reconstruction and a left-sided atrioventricular valvuloplasty. At 14-month postoperative follow-up, the patient had minimal heart failure, gained weight and adapted to biventricular circulation.
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OBJECTIVES: The aim of this study was to more accurately predict the optimal pulmonary artery (PA) reconstruction procedure (Lecompte manoeuvre or original Jatene procedure) during the arterial switch operation, we focused on the horizontal sectioning (HS) angle between the left hilum PA and the great arteries using preoperative computed tomography imaging. METHODS: We defined the HS angle α (ß) as the angle between the tangential line from the posterior (anterior) wall of the left PA at the hilum to the left anterior (right posterior) surface of the main PA and the tangential line from the left surface of the ascending aorta to the left anterior (right posterior) surface of the main PA. We identified 14 consecutive patients diagnosed with transposition of the great arteries or transposition of the great artery-type double-outlet right ventricle who underwent preoperative computed tomography imaging. The original Jatene or Lecompte procedure was used for 9 (OJ group) and 5 (L group) patients. Relationships of the great arteries of the OJ and L groups were side by side in 8 and 2 patients, oblique in 1 and 1 patient and anteroposterior in 0 and 2 patients, respectively. RESULTS: In the OJ group, ß was greater than α in all patients. The median α/ß value was 0.618. In group L, α was greater than ß in all patients. The median α/ß was 1.307. Left PA stenosis caused by stretching was not observed in the L group. Coronary obstruction was not identified in the OJ group. Left PA stenosis behind the neo-ascending aorta was observed in 1 patient in the OJ group and required reoperation. CONCLUSIONS: The HS angle may be a useful predictor of optimal intraoperative PA reconstruction during arterial switch operation, especially for side-by-side or oblique relationships.
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Operación de Switch Arterial , Transposición de los Grandes Vasos , Humanos , Lactante , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Transposición de los Grandes Vasos/diagnóstico por imagen , Transposición de los Grandes Vasos/cirugía , Constricción Patológica , Aorta/diagnóstico por imagen , Aorta/cirugíaRESUMEN
Aortic valve surgery carries increased risks in patients with an extensively calcified aorta. We describe a technique in which we maintain systemic perfusion via bilateral axillary artery perfusion in conjunction with endoaortic balloon occlusion, and limit the circulatory arrest time.
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Estenosis de la Válvula Aórtica/cirugía , Válvula Aórtica/cirugía , Oclusión con Balón/métodos , Calcinosis/cirugía , Implantación de Prótesis de Válvulas Cardíacas/métodos , Anciano , Válvula Aórtica/patología , Arteria Axilar , Paro Cardíaco Inducido , Humanos , Masculino , Perfusión , Índice de Severidad de la Enfermedad , Factores de Tiempo , Resultado del TratamientoRESUMEN
This report describes a case of Ebstein anomaly in a fetus with cardiomegaly, severe tricuspid regurgitation, pulmonary regurgitation, and retrograde ductal flow that showed a marked increase in the size of the right atrium with advancing gestational age. Elective preterm delivery was performed at 35 weeks gestation. The prostaglandin E1 infusion resulted in more pronounced systemic hypotension and acidosis secondary to circular shunt across the patent ductus arteriosus as well as pulmonary regurgitation and tricuspid regurgitation. Emergency surgical intervention consisting of main pulmonary artery ligation, ductus arteriosus ligation, central shunt creation, and plication of the right atrium without cardiopulmonary bypass was performed 4 h after birth. At the age of 16 days, the Starnes procedure was performed. The infant's postoperative course was uneventful. A fetus that has Ebstein anomaly associated with pulmonary regurgitation is at risk for circular shunt across the patent ductus arteriosus after delivery. Planned delivery and surgical intervention without delay after birth are useful for the treatment of such cases.
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Anomalía de Ebstein/cirugía , Enfermedades del Prematuro/cirugía , Adulto , Parto Obstétrico , Anomalía de Ebstein/diagnóstico por imagen , Ecocardiografía , Femenino , Humanos , Recién Nacido , Recien Nacido Prematuro , Embarazo , Nacimiento Prematuro , Ultrasonografía PrenatalRESUMEN
An incidental finding of right coronary artery compression between the dilated aortic root and the sternum was detected on follow-up cardiac catheterization after Fontan completion for tricuspid atresia with transposition of the great arteries. Although this 31-year-old man had no subjective symptoms, an exercise stress test and perfusion scan revealed ischemic changes in the inferior region. Valve-sparing aortic root replacement using the reimplantation technique proved to be an effective approach for this condition. The possibility of coronary artery compression associated with aortic root dilatation should be considered in adult patients with congenital heart disease.
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Procedimiento de Fontan , Cardiopatías Congénitas , Transposición de los Grandes Vasos , Adulto , Válvula Aórtica/cirugía , Vasos Coronarios/cirugía , Cardiopatías Congénitas/cirugía , Humanos , Masculino , ReimplantaciónRESUMEN
Few reports have described the implantation and management of an implantable ventricular assist device in patients with functional univentricular anatomy. We herein report a case of a patient who underwent Heartware ventricular assist device (Medtronic, Minneapolis, MN) implantation due to severe cardiac dysfunction and Fontan failure in the remote period after total cavopulmonary connection. Double valve replacement was subsequently required 1 year later due to repeated Fontan failure caused by the progression of aortic and atrioventricular regurgitation. The low-profile and saddle-shaped sewing cuff design of the MITRIS mitral valve (Edwards Lifesciences, Irvine, CA) were beneficial for avoiding the Heartware ventricular assist device inflow and atrioventricular valve interference in the less-dilated ventricle.
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Procedimientos Quirúrgicos Cardíacos , Cardiopatías , Corazón Auxiliar , Humanos , Válvula Mitral/cirugía , Reimplantación , Resultado del TratamientoRESUMEN
A 32-year-old man, who had developed fulminant myocarditis leading to asystole, underwent implantation of an EVAHEART 2 left ventricular assist system with a double-cuff tipless inflow cannula and a concurrent Fontan operation. Approximately 2 years after the simultaneous EVAHEART 2 implantation and the Fontan operation, the patient underwent heart transplantation. There was no device-related thromboembolism or pump malfunction under adequate antithrombotic management during the postoperative support period. Computed tomography showed no malposition of the inflow cannula irrespective of the left ventricular chamber size. Macroscopically, the left ventricular cavity of the excised heart revealed a smooth inflow ostium with appropriate intimal proliferation and without pannus or wedge thrombus formation. These findings suggest the utility of the double-cuff tipless inflow cannula for long-term clinical applications, which may lead to favorable outcomes during long-term patient management. The double-cuff tipless inflow cannula, which does not protrude into the left ventricular cavity, potentially contributes to the prevention of suction events and the collision of the inflow cannula with the interventricular septum and left ventricular free wall. Further investigation is required to confirm the role of the unique EVAHEART 2 inflow cannula in reducing thromboembolic events.
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Corazón Auxiliar , Tromboembolia , Adulto , Cánula , Fibrinolíticos , Ventrículos Cardíacos/cirugía , Corazón Auxiliar/efectos adversos , Humanos , Masculino , Tromboembolia/etiología , Tromboembolia/prevención & controlRESUMEN
BACKGROUND: While breastfeeding provides benefits for infants and the mother, many women either do not breastfeed or terminate breastfeeding earlier than recommended. The aim of this analysis was to identify factors associated with early discontinuation of breastfeeding in Japanese women. METHODS: This study used data from medical records of women delivering a singleton live birth between March 2017 and August 2019 in Iwase General Hospital, Fukushima Prefecture, Japan to assess cessation of breastfeeding by the 1-month postpartum appointment. Demographic (age at birth, and employment status), medical (parity, and physical and mental condition of the mother; and infant medical factors, such as sex, Apgar score, and jaundice, among other), and family factors (husband/partner, family members living at the same house, among others) in 734 women who had initiated breastfeeding during their delivery hospital stay were examined, and multiple logistic regression was used to determine significant predictors of early cessation of exclusive breastfeeding. RESULTS: Bivariate analysis revealed that women who were primipara, unmarried, exposed to secondhand smoke, and employed; those who smoked before pregnancy; and those who had asthma were more likely to discontinue exclusive breastfeeding than other women. Infant factors associated with discontinuation were lower birthweight, earlier gestational age, neonatal intensive care unit admission, treatment for jaundice, or lower weight gain. Multivariable analysis revealed that primiparity, passive smoking before pregnancy, maternal employment, and neonatal jaundice therapy were associated with discontinuation of breastfeeding. CONCLUSIONS: In particular, women whose partners smoked before pregnancy may need to be targeted for additional support for breastfeeding.
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Lactancia Materna , Madres , Femenino , Humanos , Lactante , Recién Nacido , Japón , Masculino , Embarazo , Estudios Retrospectivos , Factores de RiesgoRESUMEN
OBJECTIVES: A few studies have described the outcomes of systemic-to-pulmonary artery shunt (SPS) for functional single ventricular heart with extracardiac total anomalous pulmonary venous connection (TAPVC). This study aimed to examine the outcomes of SPS with extracardiac TAPVC and identify the predictors of mortality before bidirectional Glenn operation (BDG). METHODS: Medical records of 41 children with single ventricular heart and extracardiac TAPVC who underwent SPS between 1998 and 2019 were reviewed retrospectively. The median age and body weight at SPS were 36 days and 3.4 kg, respectively. Surgical outcomes and predictors of mortality were investigated. RESULTS: Four operative deaths (10%) and 10 late deaths (27%) occurred before BDG. Of all the children, 19 underwent BDG at a median of 10 months since SPS and eight are waiting for BDG. In the multivariate analysis, preoperative pulmonary venous obstruction (p = 0.01) at initial surgery was most predictive of death before BDG. Patients who underwent simultaneous SPS and TAPVC repair were younger, had high preoperative rate of pulmonary venous obstruction, and more deaths before BDG. CONCLUSIONS: Survival outcomes of SPS for SVH with extracardiac TAPVC were improved as a whole due to the increase in knowledge and technique of management SPS. However, the patients who have preoperative pulmonary venous obstruction (PVO) and need SPS and TAPVC repair concomitantly in the early postnatal period have poor outcomes and still challenging. In such a case, staged TAPVC repair and SPS may be beneficial.