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1.
BMC Cardiovasc Disord ; 22(1): 112, 2022 03 17.
Artigo em Inglês | MEDLINE | ID: mdl-35300595

RESUMO

BACKGROUND: To explore the effect of initial surgery for type I and II pulmonary atresia with intact ventricular septum (PA/IVS). METHODS: 50 children with type I PA/IVS and 50 with type II PA/IVS who had undergone initial surgery were enrolled. Children with Type I were divided into groups A (n = 25) and B (n = 25). Group A had received BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve, whereas group B had undergone BT shunt combined with PDA ligation and pulmonary valve incision. Children with type II were divided into groups C (n = 25) and D (n = 25). Group C had received BT shunt combined with PDA ligation, right ventricular outflow tract (RVOT) incision and transannular patch. Group D had undergone BT shunt combined with PDA ligation, RVOT incision, transannular patch and artificial pulmonary valve implantation. The differences in mechanical ventilation time, length of ICU stay, mortality rate, tricuspid Z value, tricuspid regurgitation, oxygen saturation, pulmonary regurgitation, McGoon ratio, pulmonary artery transvalvular pressure, survival rate were compared between groups A and B, between groups C and D respectively. RESULTS: The ventilator assistance time and length of ICU stay were greater in group C than in group D (80.96 ± 8.42 h vs. 65.16 ± 4.85 h, P = 0.045; 222.00 ± 11.72 h vs. 162.48 ± 7.91 h, P = 0.048). The pulmonary artery transvalvular pressure was significantly higher in group A than in group B at 3, 6, 12, 24 and 36 months after surgery (64.86 ± 4.13 mmHg vs. 53.04 ± 5.64 mmHg, P = 0.045; 69.47 ± 1.93 mmHg vs. 55.95 ± 4.04 mmHg, P = 0.005; 80.16 ± 3.76 mmHg vs. 73.24 ± 2.34 mmHg, P = 0.035; 62.95 ± 5.64 mmHg vs. 48.47 ± 7.44 mmHg, P = 0.04; 53.69 ± 4.89 vs. 45.77 ± 3.26, P = 0.02). Furthermore, the tricuspid Z value was significantly greater in group B than in group A at 3 and 24 months after surgery (- (1.37 ± 0.04) vs. - (1.43 ± 0.06), P = 0.03; - (0.41 ± 0.06) vs. - (0.51 ± 0.11), P = 0.02). CONCLUSIONS: The effect of BT shunt combined with PDA ligation and pulmonary valve incision is superior to BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve, and the effect of BT shunt combined with PDA ligation, RVOT incision, transannular patch and artificial pulmonary valve implantation is superior to BT shunt combined with PDA ligation, RVOT incision and transannular patch.


Assuntos
Cardiopatias Congênitas , Atresia Pulmonar , Insuficiência da Valva Pulmonar , Valva Pulmonar , Criança , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/cirurgia , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Resultado do Tratamento
2.
World J Pediatr Congenit Heart Surg ; 13(2): 178-186, 2022 03.
Artigo em Inglês | MEDLINE | ID: mdl-35238703

RESUMO

OBJECTIVE: Conotruncal anomalies can develop aortopathy and/or aortic valve (AV) disease and AV replacement (AVR) is occasionally needed. We report long-term results and examine factors affecting survival following AVR in this group. METHODS: We queried the Pediatric Cardiac Care Consortium (PCCC, US database for interventions for congenital heart diseases) to identify patients with repaired conotruncal anomalies and AVR. Long-term outcomes were provided by the PCCC, the US National Death Index, and Organ Procurement and Transplantation Network. Competing risks analysis examined outcomes following AVR (death/transplantation, reoperation) and multivariable regression analysis assessed significant factors. RESULTS: One hundred six children with repaired conotruncal anomalies underwent AVR (1982-2003). Underlying anomaly was truncus (n = 40), d-transposition (n = 22), type-B interrupted arch (n = 16), double-outlet right ventricle (n = 12), pulmonary atresia with ventricular septal defect (n = 9), tetralogy of Fallot (n = 6), corrected transposition (n = 1). 18 (17%) had prior aortic valvuloplasty (surgical = 12, percutaneous = 6). Median age at AVR was 6.9 years (interquartile range = 2.5-12.4). AV pathophysiology was regurgitation (n = 83, 78%), stenosis (n = 9, 9%), and mixed (n = 14, 15%). AVR type was mechanical (n = 72, 68%), homograft (n = 21, 20%), and Ross (n = 13, 12%). Operative mortality was 13(12%). Infant age at AVR was risk factor (odds ratio = 55, 95% confidence interval [CI] = 6-539, P = .0006). On competing risks analysis, five years after AVR, 6% died or received transplantation, 20% had reoperation. Twenty-five years transplant-free survival was 53%. Factors associated with death after hospital discharge included mitral surgery (hazards ratio [HR] = 11, 95% CI = 3-39, P = .0002), underlying defect (HR = 2, 95% CI = 1-5, P = .446). Twenty years transplant-free survival in conotruncal anomalies group was inferior to matched children undergoing AVR for congenital non-conotruncal disease (61% vs 82%, P = .0012). CONCLUSIONS: Long-term survival following AVR in children with conotruncal anomalies is inferior to that of isolated congenital AV disease and is linked to an underlying cardiac defect. Although valve type was not associated with survival, infant age was a risk factor for operative mortality. Continuous attrition and high reoperation warrant vigilant monitoring.


Assuntos
Cardiopatias Congênitas , Comunicação Interventricular , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Atresia Pulmonar , Valva Aórtica/cirurgia , Criança , Cardiopatias Congênitas/cirurgia , Comunicação Interventricular/cirurgia , Implante de Prótese de Valva Cardíaca/métodos , Humanos , Lactente , Atresia Pulmonar/cirurgia , Reoperação , Estudos Retrospectivos , Resultado do Tratamento
3.
J Card Surg ; 37(6): 1718-1719, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35338714

RESUMO

We report a case of a 5-year-old child with tetralogy of Fallot-pulmonary atresia with anomalous origin of left pulmonary artery from ascending aorta and right lung supplied by collateral arteries. This case highlights the role of preoperative CT angiography in differentiating this entity from a common arterial trunk with discontinuous pulmonary arteries.


Assuntos
Atresia Pulmonar , Tetralogia de Fallot , Anormalidades Múltiplas , Aorta/anormalidades , Aorta/diagnóstico por imagem , Aorta/cirurgia , Pré-Escolar , Humanos , Pulmão/anormalidades , Pneumopatias , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/cirurgia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia
4.
Rev Cardiovasc Med ; 23(3): 85, 2022 Mar 04.
Artigo em Inglês | MEDLINE | ID: mdl-35345252

RESUMO

OBJECTIVE: The study aims to evaluate the feasibility and effectiveness of an individualized procedure for right ventricular outflow tract (RVOT) reconstruction in pulmonary atresia with ventricular septal defect (PA-VSD). METHODS: RVOT was reconstructed using autologous pulmonary artery tissue preserved in situ as the posterior wall and a bovine jugular vein patch (BJVP) as the anterior wall in patients with PA-VSD (observation group). The size of the BJVP made from a bovine jugular vein conduit (BJVC) was individually calculated using a formula based on the child's weight and the size of the autologous pulmonary artery (the diameter of BJVC DB⁢J⁢V⁢C = Dt⁢h⁢e⁢o⁢r⁢e⁢t⁢i⁢c⁢a⁢l-W⁢z^-4π). Its effect was then compared with the conventional modified Rastelli procedure based on the BJVC (control group). RESULTS: A total of 22 patients that underwent the new procedure were simultaneously compared with the 25 patients in the control group. No deaths occurred in both groups. Notably, there were no significant differences in mechanical ventilation, ICU and postoperative residence, cardiopulmonary bypass, and aortic cross-clamp time. In the follow-up, which spanned for 8-12 years (mean 9.2 years), only four cases with moderate regurgitation were noted in the observation group without obstruction. In the control group, two patients had a conduit replacement. Three patients suffered from anastomotic stenosis, which was corrected by balloon dilatation. CONCLUSION: Individualized RVOT reconstruction with autologous pulmonary tissue preserved in situ as the posterior wall is adequate for treating PA-VSD.


Assuntos
Cardiopatias Congênitas , Comunicação Interventricular , Atresia Pulmonar , Septo Interventricular , Animais , Bovinos , Criança , Cardiopatias Congênitas/cirurgia , Defeitos dos Septos Cardíacos , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/cirurgia , Humanos , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/cirurgia , Resultado do Tratamento
6.
J Card Surg ; 37(4): 967-968, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35137983

RESUMO

BACKGROUND: A staged thoracotomy unifocalization approach has not been the dominant option over the past 20 years primarily due to the introduction of midline one-stage complete unifocalization. METHODS: In this issue of the Journal of Cardiac Surgery, van de Woestijne et al. publish their experience over the past 30 years in 39 consecutive patients with "consistent" staged unifocalization through a lateral thoracotomy in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCA). RESULTS: They report definitive repair completion in 76.3% of the patients and an overall survival after definitive repair of 96% at 20 years despite the study period ranging from 1989 to the present. CONCLUSION: Given the multiple variations one could have with PA/VSD/MPACA, a midline unifocalization approach may not always be possible. Surgeons should be familiar with the lateral thoracotomy unifocalization staged approach to PA/VSD/MAPCA.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Comunicação Interventricular , Atresia Pulmonar , Circulação Colateral , Comunicação Interventricular/cirurgia , Humanos , Lactente , Artéria Pulmonar/anormalidades , Atresia Pulmonar/cirurgia , Estudos Retrospectivos
7.
J Card Surg ; 37(4): 960-966, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35142386

RESUMO

OBJECTIVES: Pulmonary atresia (PA) with ventricular septal defect (VSD) and systemic-pulmonary collateral arteries (SPCAs) presents with variable anatomy with regard to the pulmonary vasculature, requiring personalized surgical treatment. A protocol consisting of staged unifocalization and correction was employed. METHODS: Since 1989, 39 consecutive patients were included (median age at first operation 13 months). In selected cases, a central aorto-pulmonary shunt was performed as the first procedure. Unifocalization procedures were performed through a lateral thoracotomy. Correction consisted of shunt takedown, VSD closure, and interposition of an allograft between the right ventricle and the reconstructed pulmonary artery. Echocardiographic data were obtained postoperatively and at interval follow-up. RESULTS: In 39 patients 66 unifocalization procedures were performed. Early mortality was 5%. Seven patients were considered not suitable for correction, of which four have since died. One patient is awaiting further correction. A correction was performed successfully in 28 patients. Operative mortality was 3% and late mortality was 11%. Median follow-up after the correction was 19 years. Eleven patients required homograft replacement. Freedom from conduit replacement was 88%, 73%, and 60% at 5, 10, and 15 years respectively. Right ventricular function was reasonable or good in 75% of patients. All but one patient were in NYHA Class I or II. CONCLUSIONS: After complete unifocalization 30/37 patients (81%) were considered correctable. The staged approach of PA, VSD, and SPCAs results in adequate correction and good functional capacity. RV function after correction remains reasonable or good in the majority of patients.


Assuntos
Cardiopatias Congênitas , Comunicação Interventricular , Atresia Pulmonar , Circulação Colateral , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/cirurgia , Humanos , Lactente , Artéria Pulmonar/anormalidades , Artéria Pulmonar/cirurgia , Atresia Pulmonar/cirurgia , Estudos Retrospectivos
8.
Gen Thorac Cardiovasc Surg ; 70(5): 491-494, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35098488

RESUMO

The Rastelli procedure is indicated for repair of tetralogy of Fallot (TOF) with pulmonary atresia. However, currently, there are no criteria available for concomitant surgical intervention for an enlarged ascending aorta in infancy. We report a rare case of TOF with pulmonary atresia complicated by severe enlargement of the ascending aorta in a girl aged 2 years and 5 months. We performed the Rastelli procedure with ascending aorta replacement. Pathological findings of the ascending aorta included fragmentation of elastic fibers and loss of smooth muscle cells. Postoperatively, the patient remained healthy and asymptomatic. This case indicates that ascending aortic enlargement in conotruncal anomalies may be associated with pathological lesion even in early childhood. Replacement of the diseased aorta could be considered as a concomitant procedure with definitive repair for conotruncal anomalies presenting with severe ascending aortic enlargement.


Assuntos
Transposição das Grandes Artérias , Cardiopatias Congênitas , Atresia Pulmonar , Tetralogia de Fallot , Aorta/diagnóstico por imagem , Aorta/patologia , Aorta/cirurgia , Pré-Escolar , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Hipertrofia/patologia , Atresia Pulmonar/cirurgia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia
9.
Pediatr Cardiol ; 43(4): 796-806, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-34988599

RESUMO

Tricuspid valve agenesis/atresia (TVA) is a congenital cardiac malformation where the tricuspid valve is not formed. It is hypothesized that TVA results from a failure of the normal rightward expansion of the atrioventricular canal (AVC). We tested predictions of this hypothesis by morphometric analyses of the AVC in fetal hearts. We used high-resolution MRI and ultrasonography on a post-mortem fetal heart with TVA and with tricuspid valve stenosis (TVS) to validate the position of measurement landmarks that were to be applied to clinical echocardiograms. This revealed a much deeper right atrioventricular sulcus in TVA than in TVS. Subsequently, serial echocardiograms of in utero fetuses between 12 and 38 weeks of gestation were included (n = 23 TVA, n = 16 TVS, and n = 74 controls) to establish changes in AVC width and ventricular dimensions over time. Ventricular length and width and estimated fetal weight all increased significantly with age, irrespective of diagnosis. Heart rate did not differ between groups. However, in the second trimester, in TVA, the ratio of AVC to ventricular width was significantly lower compared to TVS and controls. This finding supports the hypothesis that TVA is due to a failed rightward expansion of the AVC. Notably, we found in the third trimester that the AVC to ventricular width normalized in TVA fetuses as their mitral valve area was greater than in controls. Hence, TVA associates with a quantifiable under-development of the AVC. This under-development is obscured in the third trimester, likely because of adaptational growth that allows for increased stroke volume of the left ventricle.


Assuntos
Atresia Pulmonar , Atresia Tricúspide , Ecocardiografia , Feminino , Coração Fetal/diagnóstico por imagem , Humanos , Gravidez , Atresia Pulmonar/complicações , Atresia Tricúspide/complicações , Valva Tricúspide/diagnóstico por imagem , Ultrassonografia Pré-Natal
10.
Pediatr Cardiol ; 43(4): 878-886, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35022807

RESUMO

Branch pulmonary artery (PA) stenosis due to ductus arteriosus (DA) tissue (DA-PS) contributes to the morbidity associated with pulmonary atresia (PAtr). We sought to identify preoperative echocardiographic features predictive of DA-PS. Patients consecutively encountered with PAtr and a DA-dependent pulmonary circulation at birth who underwent intervention in our program over a 5-year period were identified and records reviewed. Preoperative echocardiograms were reviewed to identify features that predicted postoperative DA-PS. Seventy patients with PAtr met inclusion criteria and 36 (51%) had DA-PS. At preoperative echocardiography, the proximal diameter of the PA ipsilateral to the DA was smaller in those with versus without DA-PS (Z-score - 4.8 ± 1.7 vs - 1.1 ± 1.7, respectively p < 0.001). PA origins could not be imaged on the same axial plane in 21/36 (58%) with versus 2/34 (6%) without DA-PS. Patients with DA-PS had an obtuse posterior angle of the PA bifurcation compared to those without (128 ± 17° and 87 ± 21°, p < 0.001), and a posterior angle of > 100° best predicted DA-PS with a sensitivity of 97% and specificity of 76%. An abnormal PA relationship and/or an obtuse posterior bifurcation angle had a sensitivity, specificity, positive and negative predictive value for DA-PS of 78%, 94%, 90% and 86%, respectively. Finally, DA insertion was into the ipsilateral PA in 26/36 (72%) of cases with DA-PS. A smaller proximal ipsilateral PA diameter, inability to image the PAs in the same plane, a posterior PA bifurcation angle of > 100°, and insertion of the DA in the ipsilateral PA demonstrated by echo are useful in identifying patients at risk for DA-PS.


Assuntos
Permeabilidade do Canal Arterial , Canal Arterial , Cardiopatias Congênitas , Atresia Pulmonar , Estenose de Artéria Pulmonar , Canal Arterial/diagnóstico por imagem , Ecocardiografia/métodos , Humanos , Recém-Nascido , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/cirurgia , Estenose de Artéria Pulmonar/diagnóstico por imagem , Estenose de Artéria Pulmonar/etiologia , Estenose de Artéria Pulmonar/cirurgia
11.
J Card Surg ; 37(3): 682-684, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35028980

RESUMO

We present a case of a 16-year-old cyanotic male patient with ventricular septal defect, pulmonary atresia and discordant atrioventricular connections where computed tomography angiography demonstrated a large tortuous coronary-to-pulmonary collateral artery arising from the superdominant morphologically right coronary artery and coursing cranially to supply the pulmonary parenchyma.


Assuntos
Comunicação Interventricular , Atresia Pulmonar , Adolescente , Circulação Colateral , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/cirurgia , Humanos , Masculino , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/cirurgia
12.
Am J Case Rep ; 23: e933078, 2022 Jan 18.
Artigo em Inglês | MEDLINE | ID: mdl-35039470

RESUMO

BACKGROUND Dextro-transposition of the great arteries (D-TGA) with a ventricular septal defect (VSD) and pulmonary atresia is an uncommon congenital conotruncal abnormality. Surgical correction is performed using the Rastelli procedure, which includes a ventricular septal patch to direct blood from the left ventricle to the aorta and a valved conduit to connect the right ventricle to the pulmonary artery. This report is of a 34-year-old Thai man who presented with pulmonary stenosis and heart failure 24 years after surgical correction with the Rastelli procedure for congenital D-TGA, VSD, and pulmonary atresia. CASE REPORT A 34-year-old Thai man presented with dyspnea on moderate exertion. His cardiovascular examination revealed a median sternal surgical scar, parasternal heaving, a grade III systolic ejection murmur at the left upper parasternal border, and a single second heart sound. Echocardiography demonstrated degenerative calcification of a severely stenosed pulmonary valve and impaired right ventricular function. A color Doppler M-mode echocardiogram showed VSD patch leakage. A computed tomography scan with 3-dimensional heart reconstruction demonstrated a significantly stenosed branch pulmonary artery. Right and left heart catheterization confirmed the multi-site stenoses were hemodynamically significant. The patient underwent surgery for VSD closure, placement of a right-ventricle-to-pulmonary-artery conduit with a polytetrafluoroethylene graft, and pulmonary artery plasty to correct the stenosis at the branch of the pulmonary artery. CONCLUSIONS The long-term complications of the Rastelli-type operation seen for D-TGA with a VSD and pulmonary atresia included a right-ventricle-to-pulmonary-artery conduit obstruction, VSD patch leakage, and re-stenosis of the peripheral pulmonary stenosis. Multimodal imaging was informative in planning for reoperation.


Assuntos
Transposição das Grandes Artérias , Insuficiência Cardíaca , Comunicação Interventricular , Atresia Pulmonar , Estenose da Valva Pulmonar , Transposição dos Grandes Vasos , Adulto , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/cirurgia , Humanos , Lactente , Masculino , Artéria Pulmonar , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/cirurgia , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/cirurgia , Tailândia , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia
13.
Heart Lung Circ ; 31(4): 549-558, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-34654648

RESUMO

BACKGROUND: There is increasing use of catheter-based therapy as part of the neonatal treatment algorithm for pulmonary atresia with an intact ventricular septum (PAIVS). The management strategies utilised and outcomes of patients with PAIVS at our centre have not been examined. METHODS: A retrospective case series was undertaken including all infants with PAIVS born January 2009 to July 2019 (follow-up to January 2020) managed at The Children's Hospital at Westmead, New South Wales. Demographic features, anatomical substrate, management pathway and subsequent clinical outcomes were examined. RESULTS: Fifty-two (52) infants were included (male n=28, 53.8%). The right ventricular morphology was classified as normal, mildly, moderately and severely hypoplastic in 3 (5.8%), 13 (25.0%), 19 (36.5%) and 17 (32.7%) patients respectively. Thirty-seven (37) patients underwent an initial aortopulmonary (AP) shunt (surgical shunt or patent ductus arteriosus [PDA] stent). The remaining 15 patients underwent an initial intervention to decompress the right ventricle. Twenty (20) patients underwent a neonatal catheter-based intervention. An initial catheter-based intervention was more likely in the second half of the period. Sixteen (16) patients had an attempted pulmonary valve perforation, 12 as their initial procedure. Median follow-up was 62 months (range 3-119 months). Final circulation status was known in 37 patients; biventricular n=14 (37.8%), "1.5 ventricles" n=4 (10.8%), single n=19 (51.4%). There were five deaths during the period (9.6%), including two during the initial procedural admission attributed to tamponade requiring extracorporeal membrane oxygenation (ECMO) at the time of percutaneous pulmonary valve perforation. CONCLUSION: There has been an overall trend towards including catheter-based strategies in the neonatal period as part of management at our centre. Given the risk of bleeding and ECMO related to this, consideration should be given to the availability of multidisciplinary support when planning the timing of these procedures.


Assuntos
Cardiopatias Congênitas , Atresia Pulmonar , Septo Interventricular , Cateteres , Criança , Seguimentos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração , Humanos , Lactente , Recém-Nascido , Masculino , Atresia Pulmonar/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
15.
J Card Surg ; 37(2): 433-436, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34727400

RESUMO

INTRODUCTION: Common arterial trunk with aortic dominance has well-developed bilateral pulmonary arterial arborization without any essential major aortopulmonary collateral arteries (MAPCAs), whereas "solitary" arterial trunk is characterized by collateral arterial supply to all bronchopulmonary segments and absent pulmonary arteries. CASE REPORT: We report a term female neonate with common arterial trunk with aortic dominance with confluent pulmonary arteries with a large MAPCA as the sole blood supply to the lower lobe of the left lung. RESULTS: Initial diagnostic workup missed this MAPCA from the descending thoracic aorta. It was suspected during surgery due to massive left atrial return and confirmed by cardiac catheterization study in the early postoperative period and treated successfully by unifocalization. DISCUSSION: A large essential MAPCA supplying an entire lobe in the common arterial trunk with aortic dominance has not been described. It can present as heart failure and inability to wean off the ventilator in the early postoperative period following intracardiac repair. CONCLUSION: This is a case hitherto undescribed and possibly falling outside the well-entrenched classifications of the common arterial trunk and a large MAPCA could be an addition to the list of lesion modifiers. Our experience with this case underlines the importance of a thorough and open-minded approach to the initial imaging assessment of even well-described conditions.


Assuntos
Atresia Pulmonar , Persistência do Tronco Arterial , Aorta , Circulação Colateral , Feminino , Humanos , Lactente , Recém-Nascido , Pulmão , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia
16.
J Card Surg ; 37(2): 455-457, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34751994

RESUMO

BACKGROUND: Absent pulmonary valve (PV) is a rare and severe variant seen in only 3% to 6% of patients with tetralogy of Fallot (TOF). Fetuses with this combined condition who survive through birth typically need intervention in infancy or early childhood because of respiratory distress, heart failure, or failure to thrive. DISCUSSION: Here, we describe the case of a 37 year old gentleman who presented with a diagnosis of TOF with absent PV, hugely dilated right and left pulmonary arteries and an aneurysmal right ventricular outflow. CONCLUSION: TOF with absent PV surviving into adulthood is a rarity. An aneurysmal right ventricular outflow tract in an unrepaired case of TOF has not been described before. Massively dilated left and right pulmonary arteries as in this case can cause significant and even life-threatening airway compromise, thus necessitating early surgical repair.


Assuntos
Atresia Pulmonar , Valva Pulmonar , Tetralogia de Fallot , Adulto , Pré-Escolar , Humanos , Masculino , Artéria Pulmonar , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia
17.
Pediatr Cardiol ; 43(2): 435-442, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34559262

RESUMO

This retrospective cohort study aimed to explore the long-term outcomes of an individualized strategy in patients with pulmonary atresia and intact ventricular septum (PAIVS). We analyzed survival and reintervention rates and identified risk factors for outcomes in patients with PAIVS treated based on individual right heart structures between 1979 and 2019. Ninety-five patients were included in this study. The z-scores of the pulmonary annulus, tricuspid annulus, and right ventricular end-diastolic volume were - 3.30 (- 15.15 to 1.83), - 0.70 (- 4.65 to 2.33), and - 1.51 (- 6.35 to 1.18), respectively. Right ventricular-dependent coronary circulation occurred in 15% of the patients. Among the 63 patients attempting biventricular strategy at first, 55 patients achieved biventricular circulation, 3 patients had one-and-a-half circulation, and 4 patients died perioperatively. Among the 33 patients attempting univentricular strategy at first, 10 patients died before the completion of Fontan operation, 17 patients (48%) accomplished Fontan operation, and 5 patients waited for Fontan operation. In one patient, conversion to biventricular circulation occurred. During the follow-up period of 720 person-years, the 20-year survival rate was significantly higher in patients with biventricular circulation than in those patients with univentricular circulation (93% vs. 67%, P < 0.001). Freedom from reintervention rates at 20 years was significantly lower in patients with biventricular circulation than in those patients with univentricular circulation (29% vs. 72%, P < 0.001). The pulmonary annulus z-score was an independent risk factor for reintervention in patients with biventricular circulation. Patients with biventricular circulation had an acceptable survival rate, but a high reintervention rate. Meanwhile, patients with univentricular circulation had high mortality before the completion of Fontan operation, although the reintervention rate was relatively low.


Assuntos
Cardiopatias Congênitas , Atresia Pulmonar , Septo Interventricular , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Humanos , Atresia Pulmonar/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Septo Interventricular/diagnóstico por imagem , Septo Interventricular/cirurgia
18.
Catheter Cardiovasc Interv ; 99(4): 1129-1137, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-34800077

RESUMO

Achieving an optimal surgical result in patients with major aortopulmonary collateral arteries (MAPCAs) requires a thorough preoperative evaluation of the anatomy and physiology of the pulmonary circulation. This review provides a detailed description of diagnostic catheterization in patients with MAPCAs, including a summary of catheterization techniques, an overview of commonly used terms, and a review of MAPCA and pulmonary artery angiographic anatomy.


Assuntos
Cardiopatias Congênitas , Atresia Pulmonar , Tetralogia de Fallot , Cateterismo , Criança , Circulação Colateral , Humanos , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Atresia Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Resultado do Tratamento
19.
Heart Vessels ; 37(1): 142-151, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34089363

RESUMO

Absent pulmonary valve (APV) syndrome with tricuspid atresia or tricuspid stenosis (TA/TS) is an extremely rare malformation recently reported as a variant of APV with intact ventricular septum (VS). The condition, however, has univentricular physiology and unique structural and clinical features. The purpose of this study was to update the current knowledge about this condition by describing long-term outcomes of three new cases and reviewing the available literatures. A systematic literature search was performed to collect clinical and anatomical data of APV with TA/TS. Institutional medical records were retrospectively reviewed to identify APV with TA/TS patients. In a total of 62 (59 reported and 3 new) cases, patent ductus arteriosus was present in 98% of APV patients with TA/TS. A large ventricular septal defect, dilatation of the pulmonary arteries, which is typically found in APV with tetralogy of Fallot, and respiratory distress at birth were rarely reported. Most of the recent cases were successfully managed by the Glenn or Fontan procedure. Coronary artery anomaly and ventricular arrhythmia were more frequently reported as the cause of death or severe neurological sequelae (9/16 and 3/8, respectively). Additional surgical intervention was required in the mid/long-term period in three cases due to left-ventricular outflow obstruction and in two due to aortic dilatation. The Fontan and Glenn procedures improved the survival in the last two decades. In addition to coronary artery anomaly and ventricular arrhythmia, left-ventricular outflow tract obstruction and aortic dilatation should be carefully monitored.


Assuntos
Valva Pulmonar , Atresia Tricúspide , Constrição Patológica , Humanos , Atresia Pulmonar , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Estudos Retrospectivos
20.
Cardiol Young ; 32(2): 236-243, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34024296

RESUMO

INTRODUCTION: CHD affects over 1 million children in the United States. Studies show decreased mortality from CHD with newborn cardiac screening. California began a screening programme on 1 July, 2013. We evaluated the effect of mandatory screening on surgical outcomes at Loma Linda University Children's Hospital since 1 July, 2013. METHODS: We evaluated all infants having congenital heart surgery at Loma Linda University Children's Hospital between 1 July, 2013 and 31 December, 2018. Primary target diagnoses include hypoplastic left heart syndrome, pulmonary atresia with intact ventricular septum, tetralogy of Fallot, total anomalous pulmonary venous return, transposition of the great arteries, tricuspid atresia, and truncus arteriosus. Secondary target diagnoses include aortic coarctation, double outlet right ventricle, Ebstein anomaly, interrupted aortic arch, and single ventricle. Patients were stratified by timing of diagnosis (pre-screen, screen positive, and screen negative). Primary end points were post-operative length of stay, operative mortality, absolute mortality, and actuarial survival. RESULTS: The cohort included 274 infants. Of these, 79% were diagnosed prior to screening (46% prenatally). Only 38% of those screened were positive, with 13% of the cohort having a "missed diagnosis." CONCLUSIONS: Primary targets were more likely to be diagnosed by screening (53%), while secondary targets were unlikely to be diagnosed by screening (10%) (p = 0.004). Outcomes such as length of stay, operative mortality, and actuarial survival were not different based on timing of diagnosis (p > 0.05). Despite late diagnosis, those not diagnosed until after screening did not have adverse outcomes.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas , Atresia Pulmonar , Transposição dos Grandes Vasos , Criança , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Recém-Nascido , Triagem Neonatal
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