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1.
Cardiol Young ; : 1-8, 2024 Apr 04.
Artigo em Inglês | MEDLINE | ID: mdl-38572557

RESUMO

BACKGROUND: Invasive haemodynamics are often performed for initiating and guiding pulmonary artery hypertension therapy. Little is known about the predictive value of invasive haemodynamic indices for long-term outcomes in children with pulmonary artery hypertension. We aimed to evaluate invasive haemodynamic data to help predict outcomes in paediatric pulmonary artery hypertension. METHODS: Patients with pulmonary artery hypertension who underwent cardiac catheterisation (2006-2019) at a single centre were included. Invasive haemodynamic data from the first cardiac catheterisation and clinical outcomes were reviewed. The combined adverse outcome was defined as pericardial effusion (due to right ventricle failure), creation of a shunt for pulmonary artery hypertension (atrial septal defect or reverse Pott's shunt), lung transplant, or death. RESULTS: Among 46 patients with a median [interquartile range (IQR)] age of 13.2 [4.1-44.7] months, 76% had CHD. Median mean pulmonary artery pressure was 37 [28-52] mmHg and indexed pulmonary vascular resistance was 6.2 [3.6-10] Woods units × m2. Median pulmonary artery pulsatility index was 4.0 [3.0-4.7] and right ventricular stroke work index was 915 [715-1734] mmHg mL/m2. After a median follow-up of 2.4 years, nine patients had a combined adverse outcome (two had a pericardial effusion, one underwent atrial level shunt, one underwent reverse Pott's shunt, and six died). Patients with an adverse outcome had higher systolic and mean pulmonary artery pressures, higher diastolic and transpulmonary pressure gradients, higher indexed pulmonary vascular resistance, higher pulmonary artery elastance, and higher right ventricular stroke work index (p < 0.05 each). CONCLUSION: Invasive haemodynamics (especially mean pulmonary artery pressure and diastolic pressure gradient) obtained at first cardiac catheterisation in children with pulmonary artery hypertension predicts outcomes.

2.
J Innov Card Rhythm Manag ; 15(1): 5739-5743, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38304090

RESUMO

Pacemaker implantation in neonates can be challenging due to their small size. Even pulse generators adapted for pediatric patients, such as the Microny device (Abbott, Chicago, IL, USA), are proportionately large in comparison to the size of the smallest newborns. Due to anatomic considerations, such as small vascular and ventricular sizes, leadless pacemakers and transvenous implantation in the youngest neonatal population remain unsuitable. Even so, the desire for leadless devices has prompted the industry to create the smallest pacemakers available. Adapting the smaller Micra™ transcatheter leadless pacing system (Medtronic, Minneapolis, MN, USA) for an epicardial pacing application may be advantageous to the smallest patients. This case illustrates the use of a Micra™ device modified with a header block to serve as the pulse generator in a ventricular epicardial pacing system for a 1-day-old, 2.68-kg patient with complete heart block.

3.
Echocardiography ; 41(2): e15766, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38340258

RESUMO

BACKGROUND: A previous multicenter study showed that longitudinal changes in standard cardiac functional parameters were associated with the development of cardiomyopathy in childhood cancer survivors (CCS). Evaluation of the relationship between global longitudinal strain (GLS) changes and cardiomyopathy risk was limited, largely due to lack of quality apical 2- and 3-chamber views in addition to 4-chamber view. We sought to determine whether apical 4-chamber longitudinal strain (A4LS) alone can serve as a suitable surrogate for GLS in this population. METHODS: A4LS and GLS were measured in echocardiograms with acceptable apical 2-, 3-, and 4-chamber views. Correlation was evaluated using Pearson and Spearman coefficients, and agreement was evaluated with Bland-Altman plots. The ability of A4LS to identify normal and abnormal values compared to GLS as the reference was evaluated. RESULTS: Among a total of 632 reviewed echocardiograms, we identified 130 echocardiograms from 56 patients with adequate views (38% female; mean age at cancer diagnosis 8.3 years; mean follow-up 9.4 years). Correlation coefficients between A4LS and GLS were .89 (Pearson) and .85 (Spearman), with Bland-Altman plot of GLS-A4LS showing a mean difference of -.71 ± 1.8. Compared with GLS as the gold standard, A4LS had a sensitivity of 86% (95% CI 79%-93%) and specificity of 82% (69%-95%) when using normal range cutoffs and 90% (82%-97%) and 70% (58%-81%) when using ±2 standard deviations. CONCLUSION: A4LS performs well when compared with GLS in this population. Given the more recent adoption of apical 2- and 3-chamber views in most pediatric echocardiography laboratories, A4LS is a reasonable stand-alone measurement in retrospective analyses of older study cohorts and echocardiogram biorepositories.


Assuntos
Sobreviventes de Câncer , Cardiomiopatias , Neoplasias , Disfunção Ventricular Esquerda , Criança , Feminino , Humanos , Masculino , Ecocardiografia , Neoplasias/complicações , Estudos Retrospectivos , Volume Sistólico , Função Ventricular Esquerda , Adolescente
4.
5.
World J Pediatr Congenit Heart Surg ; 14(2): 227-230, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36952283

RESUMO

A 21-month-old boy was diagnosed with partial anomalous pulmonary venous connection, with the right upper pulmonary veins draining to the superior vena cava (SVC). Intraoperatively, it became evident that the right upper pulmonary veins connected to the azygous vein before draining to the SVC.


Assuntos
Veias Pulmonares , Síndrome de Cimitarra , Masculino , Humanos , Lactente , Veia Cava Superior/cirurgia , Veia Cava Superior/anormalidades , Toracotomia , Veia Ázigos/cirurgia , Síndrome de Cimitarra/diagnóstico por imagem , Síndrome de Cimitarra/cirurgia , Veias Pulmonares/cirurgia , Veias Pulmonares/anormalidades
6.
Cardiol Young ; 33(10): 2034-2040, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36514992

RESUMO

BACKGROUND: Paediatric studies have shown serum N-terminal pro b-type natriuretic peptide levels to be a valuable tool in the surveillance of myocardial function and an early biomarker for rejection in transplant patients. The correlation between low mean right atrial pressure and increased inferior vena cava collapsibility index is well studied in adults. Our study aims to assess correlation between non-invasive measurements (serum N-terminal pro b-type natriuretic peptide, inferior vena cava dimensions collapsibility, tricuspid regurgitation, and left ventricular remodelling index to invasive mean right atrial pressure in paediatric heart transplant patients). METHODS: A single centre, retrospective chart review of the paediatric transplant patients from 0 to 21 years of age was performed between 2015 and 2017. Thirty-nine patients had complete data which includes cardiac catheterisation, transthoracic echocardiogram, and serum N-terminal pro b-type natriuretic peptide levels done within a two weeks of interval. RESULTS: A higher inferior vena cava collapsibility index correlated with a lower mean right atrial pressure (r = -0.21, p = 0.04) and a larger inferior vena cava diameter in expiration indexed to body surface area (IVCmax/BSA0.5) correlated with a higher mean right atrial pressure (r = 0.29, p = 0.01). There was a correlation between elevated N-terminal pro b-type natriuretic peptide and inferior vena cava collapsibility index (r = -0.38, p = 0.0001), IVCmax/BSA0.5 (r = 0.25, p = 0.0002), and mean right atrial pressure (r = 0.6, p = 0.0001). CONCLUSION: Serum N-terminal pro b-type natriuretic peptide levels correlated to non-invasive measurements (inferior vena cava collapsibility index and IVCmax/BSA0.5) and to the invasive mean right atrial pressure. Non-invasive (IVC-CI IVCmax/BSA0.5) correlates with elevated mean right atrial pressure in this population. Together, these may serve as a reliable surveillance tool in assessing right heart filling pressures and cardiac function within the paediatric heart transplant patient.


Assuntos
Transplante de Coração , Peptídeo Natriurético Encefálico , Adulto , Humanos , Criança , Estudos Retrospectivos , Ecocardiografia , Pressão Atrial , Veia Cava Inferior/diagnóstico por imagem
7.
World J Pediatr Congenit Heart Surg ; 14(2): 185-193, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36511245

RESUMO

BACKGROUND: Anomalies of the pulmonary origin of the pulmonary arteries are uncommon; however, the true incidence is unknown and may be underestimated. We sought to review our experience with this unusual pathology and examine its surgical implications. METHODS: We reviewed medical records between 2011 and 2022. RESULTS: A total of 24 patients were identified. Genetic syndromes were present in 12 patients (50%). The patients were divided into two main groups. Those with septal defects (14 patients,58.3%), and those without (10 patients, 41.7%). Aortic arch hypoplasia with/without coarctation was present in nine patients (37.5%). Pulmonary arterial branch hypoplasia and/or obstruction was present in six patients (25%) with resultant preoperative systemic or suprasystemic right ventricular pressure. Four patients (16.7%) underwent pulmonary artery branch and/or right ventricular outflow tract interventions prior to surgery. All patients underwent surgical interventions for their associated cardiac defects. The crossed pulmonary arteries were uncrossed for those four patients (16.7%) who required concomitant pulmonary arterioplasties and pulmonary arterial branch rehabilitation and who had elevated right ventricular pressures preoperatively. No early or late mortalities. One Alagille's syndrome underwent liver transplant prior to discharge. Late transcatheter interventions were needed in four (16.7%) during the follow-up period, while four patients required repeat aortic arch augmentation for recurrent arch obstruction. CONCLUSIONS: Anomalous origin of the pulmonary arteries from the pulmonary trunk including crossed pulmonary arteries is an uncommon anomaly that may be underrecognized. It can occur in isolation or in association with other heart defects. Recognition of this malpositional anomaly is important as it has specific surgical and/or transcatheter implications.


Assuntos
Coartação Aórtica , Cardiopatias Congênitas , Defeitos dos Septos Cardíacos , Hipertensão Pulmonar , Humanos , Lactente , Artéria Pulmonar/cirurgia , Artéria Pulmonar/anormalidades , Hipertensão Pulmonar/complicações , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/complicações , Coartação Aórtica/complicações , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/cirurgia , Defeitos dos Septos Cardíacos/complicações
8.
Ann Thorac Surg ; 115(4): 1000-1007, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36174775

RESUMO

BACKGROUND: The Inspiris Resilia (Edwards Lifesciences LLC) bioprosthesis has gained widespread use in the aortic position; however no robust data are available about its use in the pulmonary position. METHODS: We reviewed our outcomes for the Inspiris Resilia in the pulmonary position between August 2019 and October 2021. RESULTS: Twenty-seven patients (15 female patients [56%]; mean age, 22.26 ± 14.99 years) were included. The most common original pathology was tetralogy of Fallot (13 patients, 48%). Five patients (18.5%) had prior transcatheter interventions. The mean right ventricular end-diastolic volume index was 164.25 ± 45.1 mL/m2. Sternotomy or repeat sternotomy was the most common approach (21 patients, 77.8%). The standard technique for pulmonary valve replacement was used in 22 patients (81.5%), whereas the prosthesis was implanted as a conduit in the remaining 5 (18.5%). Trivial to mild prosthetic regurgitation was present in 6 patients (22.2%) at the time of discharge. There was no early mortality. Follow-up was complete in all patients (mean, 16 ± 8 months), with no late mortality or late reoperations. New prosthetic regurgitation developed in 13 patients (48%), all of whom underwent replacement with the standard surgical technique. No regurgitation occurred in the conduit cases. This progressed to moderate regurgitation in 6 patients (22%) and severe in 3 (11%). Three patients (11%) underwent transcatheter valve-in-valve after their surgical pulmonary valve replacement. CONCLUSIONS: The early data regarding the Inspiris Resilia bioprosthesis use in the pulmonary position is concerning. The prosthesis design may not be suitable for low-pressure circulation, or modification of its implantation technique may be needed.


Assuntos
Bioprótese , Procedimentos Cirúrgicos Cardíacos , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Humanos , Feminino , Criança , Adolescente , Adulto Jovem , Adulto , Implante de Prótese de Valva Cardíaca/métodos , Resultado do Tratamento , Desenho de Prótese
9.
J Cardiovasc Dev Dis ; 11(1)2023 Dec 28.
Artigo em Inglês | MEDLINE | ID: mdl-38248878

RESUMO

BACKGROUND: Phrenic nerve injury is a devastating complication that results in significant morbidity and mortality. We developed a novel technique to localize the phrenic nerve and evaluate its success. METHODS: Two groups of children underwent repeat sternotomy for a variety of indications. Group I (69 patients, nerve stimulator) and Group II (78 patients, no nerve stimulator). RESULTS: There was no significant difference in the mean age and weight between the two groups: (6.4 ± 6.5 years vs. 5.6 ± 6.4 years; p = 0.65) and (25.2 ± 24.1 vs. 22.6 ± 22.1; p = 0.69), respectively. The two groups were comparable in the following procedures: pulmonary conduit replacement, bidirectional cavopulmonary anastomosis, aortic arch repair, and Fontan, while Group I had more pulmonary arterial branch reconstruction (p = 0.009) and Group II had more heart transplant patients (p = 0.001). There was no phrenic nerve injury in Group I, while there were 13 patients who suffered phrenic nerve injury in Group II (p < 0.001). No early mortality in Group I, while five patients died prior to discharge in Group II. Eleven patients underwent diaphragm plication in Group II (p = 0.001). The mean number of hours on the ventilator was significantly higher in Group II (137.3 ± 324.9) compared to Group I (17 ± 66.9), p < 0.001. Group II had a significantly longer length of ICU and hospital stays compared to Group I (p = 0.007 and p = 0.006 respectively). CONCLUSION: Phrenic nerve injury in children continues to be associated with significant morbidities and increased length of stay. The use of intraoperative phrenic nerve stimulator can be an effective way to localize the phrenic nerve and avoid its injury.

10.
J Card Surg ; 37(12): 4526-4533, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36321701

RESUMO

BACKGROUND: Cor Triatriatum Sinister (CTS) is a rare congenital anomaly with an estimated incidence of 0.4%, resulting from abnormal left atrial septation. It may present in isolation or in association with other heart defects. High percentage of patients require surgery with low mortality and recurrence rates. CTS has been classified in the past however we aim to describe a case series with more comprehensive and inclusive classification. METHODS: This was a single-center retrospective cohort study of 16 children with the diagnosis of CTS between 2000 and 2020. Medical records were reviewed for clinical presentations, hospital, and postoperative courses. RESULTS: Sixteen patients (63% female), with a median age at diagnosis of 4.3 months, five (31%) were neonates. Six (38%) had isolated CTS, two (13%) with functional single ventricle (SV), and the remaining eight patients (50%) had other associated heart defects (septal defects in three, coarctation of the aorta in another three, and anomalous pulmonary venous connections in three). Eight patients (50%) presented with obstructed CTS. Twelve patients (75%) underwent surgical intervention. Mortality occurred in three patients (19%) with two surgical (one with total anomalous pulmonary venous connection and another with SV) and one nonsurgical (septal defect with Fanconi anemia). The surgical median follow up was 4.7 years. Recurrence of the membrane occurred in two patients (17%). CONCLUSIONS: This study showed good long-term outcomes for patients with isolated and complex CTS. Complete and proper classification of CTS ensures appropriate diagnosis, surgical planning, and better family counseling which may correlate with outcomes.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Coração Triatriado , Recém-Nascido , Criança , Humanos , Feminino , Lactente , Masculino , Coração Triatriado/diagnóstico , Coração Triatriado/cirurgia , Estudos Retrospectivos , Átrios do Coração , Procedimentos Cirúrgicos Cardíacos/métodos , Resultado do Tratamento
11.
J Card Surg ; 37(12): 4487-4491, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36208106

RESUMO

BACKGROUND: Warden procedure has been described for repair of partial anomalous pulmonary venous connections inserted high into the superior vena cava. In the current report, we describe a new modification. METHODS: A total of six patients (five females, 83%) with a mean age of 18.8 ± 10.8 years, underwent a modified Warden procedure with a descending thoracic aortic (DTA) homograft connected to the free wall of the right atrium, rather than to the right atrial appendage in the period between June 2021 and July 2022. RESULTS: All patients had evidence of enlarged right-sided cardiac chambers. Two patients had intact interatrial septum (33.3%), while three patients had sinus venosus (50%) and one had secundum atrial septal defects (16.7%). The procedure was performed via minimally invasive right thoracotomy in half of the patients. Patients were extubated in the operating room or soon after. No early mortality or reoperation. One patient required concomitant aortic root replacement and one needed repair of anomalous left upper pulmonary vein connection to the left innominate vein. Follow-up was complete in all patients with a mean of 6.2 ± 4.6 months. No late mortality or reoperation. All patients were discharged on antiplatelet therapy. Follow-up imaging studies showed satisfactory repair with widely patent superior vena caval and pulmonary venous pathways. CONCLUSIONS: The use of DTA homograft to facilitate superior vena caval translocation in the modified Warden procedure appears to be beneficial as it decreases tension on the anastomosis and avoids the need for anticoagulation.


Assuntos
Comunicação Interatrial , Veias Pulmonares , Feminino , Humanos , Criança , Adolescente , Adulto Jovem , Adulto , Veia Cava Superior/cirurgia , Veia Cava Superior/anormalidades , Comunicação Interatrial/cirurgia , Veias Pulmonares/anormalidades , Átrios do Coração , Aloenxertos
12.
Pediatr Cardiol ; 42(4): 918-925, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33590324

RESUMO

Pericardiocentesis is traditionally performed using a subxiphoid approach. Hepatomegaly or loculated and noncircumferential effusions warrant nonstandard approaches to drain effusions; echocardiographic guidance has made these less traditional, non-subxiphoid approaches feasible. The study is aimed at comparing clinical outcomes of the subxiphoid and non-subxiphoid approaches to percutaneous pericardiocentesis in a pediatric population. This is a retrospective chart review of all children undergoing percutaneous pericardiocentesis from August 2008 to December 2019 at a single-center. A total of 104 patients underwent echocardiography-guided pericardiocentesis during the timeframe. Additionally, fluoroscopy was also used in 80 patients. Hematopoietic stem cell transplantation was the most common underlying diagnosis (n = 53, 50.9%). A non-subxiphoid approach was used in 58.6% (n = 61) of patients. The fifth and sixth intercostal spaces were the most commonly used (n = 17 each). The non-subxiphoid group tended to be older (95.9 vs. 21.7 months, p = 0.006) and weighed more (23.6 vs. 11.2 kgs, p = 0.013) as compared to the subxiphoid group. Non-subxiphoid approach was associated with shorter procedure times (21 vs. 37 min, p = 0.005). No major complications were seen. Five minor complications occurred and were equally distributed in the two groups. Complications were more likely in younger patients (p = 0.047). The technique and anatomic approach to pericardiocentesis, and the location or size of effusion did not influence the risk of complications. Echocardiography-guided percutaneous pericardiocentesis in children was associated with low complication rates in this single-center pediatric experience. The use of a non-traditional, non-subxiphoid approach was associated with shorter procedure times and did not significantly affect complication rates.


Assuntos
Derrame Pericárdico/cirurgia , Pericardiocentese/métodos , Criança , Pré-Escolar , Drenagem/métodos , Ecocardiografia/métodos , Feminino , Fluoroscopia/métodos , Transplante de Células-Tronco Hematopoéticas , Humanos , Lactente , Masculino , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Resultado do Tratamento
13.
Echocardiography ; 38(2): 296-303, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33486820

RESUMO

BACKGROUND: Retrospective multicenter research using echocardiograms obtained for routine clinical care can be hampered by issues of individual center quality. We sought to evaluate imaging and patient characteristics associated with poorer quality of archived echocardiograms from a cohort of childhood cancer survivors. METHODS: A single blinded reviewer at a central core laboratory graded quality of clinical echocardiograms from five centers focusing on images to derive 2D and M-mode fractional shortening (FS), biplane Simpson's ejection fraction (EF), myocardial performance index (MPI), tissue Doppler imaging (TDI)-derived velocities, and global longitudinal strain (GLS). RESULTS: Of 535 studies analyzed in 102 subjects from 2004 to 2017, all measures of cardiac function could be assessed in only 7%. While FS by 2D or M-mode, MPI, and septal E/E' could be measured in >80% studies, mitral E/E' was less consistent (69%), but better than EF (52%) and GLS (10%). 66% of studies had ≥1 issue, with technical issues (eg, lung artifact, poor endocardial definition) being the most common (33%). Lack of 2- and 3-chamber views was associated with the performing center. Patient age <5 years had a higher chance of apex cutoff in 4-chamber views compared with 16-35 years old. Overall, for any quality issue, earlier era of echo and center were the only significant risk factors. CONCLUSION: Assessment of cardiac function using pooled multicenter archived echocardiograms was significantly limited. Efforts to standardize clinical echocardiographic protocols to include apical 2- and 3-chamber views and TDI will improve the ability to quantitate LV function.


Assuntos
Disfunção Ventricular Esquerda , Função Ventricular Esquerda , Adolescente , Adulto , Pré-Escolar , Estudos de Coortes , Ecocardiografia , Humanos , Estudos Retrospectivos , Volume Sistólico , Disfunção Ventricular Esquerda/diagnóstico por imagem , Adulto Jovem
14.
Am J Obstet Gynecol MFM ; 2(1): 100078, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-33345990

RESUMO

BACKGROUND: Chromosomal microarray analysis has emerged as a primary diagnostic tool in prenatally diagnosed congenital heart disease and other structural anomalies in clinical practice. OBJECTIVE: Our study aimed to investigate the diagnostic yield of microarray analysis as a first-tier test for chromosomal abnormalities in fetuses with both isolated and nonisolated congenital heart disease and to identify the association of different pathogenic chromosomal abnormalities with different subgroups of congenital heart disease. STUDY DESIGN: Retrospective data from 217 pregnancies that were diagnosed with congenital heart disease between 2011 and 2016 were reviewed. All pregnancies were investigated with the use of microarray analysis during the study period. Classification of chromosomal abnormalities was done based on American College of Medical Genetics and Genomics guidelines into (1) pathogenic chromosomal abnormalities that included numeric chromosomal abnormalities (aneuploidy and partial aneuploidy) and pathogenic copy number variants (22q11.2 deletion and other microdeletions/microduplications), (2) variants of uncertain significance, and (3) normal findings. RESULTS: Our study found a detection rate for pathogenic chromosomal abnormalities (numeric and pathogenic copy number variants) of 36.9% in pregnancies (n=80) that were diagnosed prenatally with congenital heart disease who underwent invasive testing with chromosomal microarray. The detection rate for numeric abnormalities was 29.5% (n=64) and for pathogenic copy number variants was 7.4% (n=16) of which 4.2% were 22q11.2 deletion and 3.2% were other pathogenic copy number variants, most of which theoretically could have been missed by the use of conventional karyotype alone. Pathogenic copy number variants were most common in conotruncal defects (19.6%; 11/56) that included 42.9% in cases of interrupted aortic arch, 23.8% in cases of tetralogy of Fallot, 13.3% in cases of transposition of the great arteries, and 8.3% in cases of double outlet right ventricle. Of these changes, 81.8% were 22q11.2 deletion, and 18.2% were other microdeletions/microduplications. After conotruncal defects, pathogenic copy number variants were most common in right ventricular outflow tract and left ventricular outflow tract groups (8% and 2.2%, respectively) in which none were 22q11.2 deletion. Pathogenic chromosomal abnormalities (numeric and pathogenic copy number variants) detected by chromosomal microarray analysis were significantly more common in the nonisolated congenital heart disease group (64.5%; n=49) compared with the isolated group (22%; n=31; P<.001). CONCLUSION: In pregnancies that were diagnosed with congenital heart disease and had undergone diagnostic genetic testing, our study showed that chromosomal microarray analysis has an added value in the detection of pathogenic chromosomal abnormalities compared with conventional karyotype, particularly in cases of pathogenic copy number variants. This yield is influenced not only by the type of congenital heart disease but also by the presence of extracardiac anomalies.


Assuntos
Cardiopatias Congênitas , Transposição dos Grandes Vasos , Feminino , Cardiopatias Congênitas/diagnóstico , Humanos , Cariotipagem , Análise em Microsséries , Gravidez , Estudos Retrospectivos
15.
Echocardiography ; 37(9): 1449-1453, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32875642

RESUMO

BACKGROUND: Intraoperative vascular imaging is a useful tool to evaluate coronary ostia in congenital heart patients with suboptimal preoperative imaging findings. Additionally, vascular imaging has potential value for visualization of the ascending aortic intima media thickness (IMT) in certain clinical scenarios. This study sought to evaluate the feasibility of intraoperative vascular imaging of coronary ostia and IMT during congenital heart surgery. METHODS: We describe the technique for performance of intraoperative vascular imaging by a pediatric cardiologist using a high-resolution linear sequential array transducer. RESULTS: Intraoperative vascular imaging was obtained on seven patients. Coronary ostia were normal in all except one. This patient had congenital stenosis of the left coronary ostium discovered during intraoperative imaging and confirmed by the surgeon. In another patient with Williams syndrome, the IMT was noted to be prominent. CONCLUSIONS: It is feasible to perform intraoperative vascular imaging in less than 5 minutes of operator's time and provides superior visualization of the coronary ostia and IMT.


Assuntos
Espessura Intima-Media Carotídea , Cardiopatias Congênitas , Aorta/diagnóstico por imagem , Aorta/cirurgia , Criança , Estudos de Viabilidade , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Humanos
16.
Pediatr Cardiol ; 41(7): 1425-1431, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32567011

RESUMO

Long-term right ventricular pacing is associated with left ventricular dysfunction and cardiomyopathy, particularly in pediatric patients and those with congenital heart disease (CHD). Research has shown that pacing-induced cardiomyopathy can be reversed with nonselective or selective His bundle pacing in adults, however, the information available about the use of this type of therapy in pediatrics and CHD is scarce. We performed a retrospective chart review of all the cases of His or left bundle pacing at the University of Minnesota, division of Pediatric Cardiology from January of 2019 to April of 2020. Parametric data are presented as mean ± standard deviation. Non-parametric data are presented as median value with interquartile ranges. Eight patients, ages 8 to 18 years (median of 11.5) and weight from 21.5 to 81.6 kg (median of 40 kg) underwent this procedure successfully. The most common structural heart disease was a repaired peri-membranous ventricular septal defect. Three patients (37.5%) had selective and three (37.5%) had nonselective His bundle pacing, and two patients (25%) had left bundle pacing. There were two cases of pacing-induced cardiomyopathy and each had a 14% and 16% improvement of the ejection fraction after nonselective His bundle pacing. There were no procedural complications. Selective and nonselective His bundle, as well as left bundle pacing may be a feasible procedure in pediatric patients with and without CHD. This procedure may improve pacing-induced cardiomyopathy in this population.


Assuntos
Fascículo Atrioventricular/cirurgia , Estimulação Cardíaca Artificial/métodos , Adolescente , Fascículo Atrioventricular/fisiopatologia , Cardiomiopatias/fisiopatologia , Criança , Eletrocardiografia , Feminino , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pediatria , Estudos Retrospectivos
17.
Pediatr Cardiol ; 41(5): 972-978, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32356015

RESUMO

There are limited data on the relation between congenital heart disease (CHD) and preterm birth (PTB). We aimed to estimate the risk of PTB in newborns with CHD, to study associations and risk factors (modifiable and non-modifiable) as well as investigate postnatal outcomes. This was a retrospective cohort study of 336 pregnancies diagnosed with CHD between 2011 and 2016. Groups consisted of those delivered at or after 37 weeks, and those who delivered prior to 37 weeks. Collected data included maternal and fetal characteristics as well postnatal outcomes. Complete data were obtained from 237 singleton pregnancies. The overall proportion of PTB was 23.2% for all CHD, of which 38.2% were spontaneous PTB which was almost unchanged after excluding extracardiac anomalies and pathogenic chromosomal abnormalities. Significant non-modifiable risk factors were pregnancy-related HTN disorders (P < 0.001), fetal growth restriction (P = 0.01), and pathogenic chromosomal abnormalities (P = 0.046). Significant PTB modifiable risk factors included prenatal marijuana use (P = 0.01). Pregnancies delivered at 37-38 weeks had significantly more newborns with birthweight < 2500 g (P < 0.001), required more pre-operative NICU support including intubation (P = 0.049), vasopressors (P = 0.04), prostaglandins (P = 0.003), antibiotics (P = 0.01), and had longer hospital stay (P = 0.001) than those delivered at ≥ 39 weeks. Prenatally diagnosed pregnancies with CHD had higher PTB rate compared to the general population, with spontaneous PTB comprising 38.2% of these preterm deliveries. Most PTB risk factors were non-modifiable, however, significant modifiable factors included marijuana use in pregnancy. Outcomes were favorable in neonates delivered at or beyond 39 weeks.


Assuntos
Cardiopatias Congênitas/epidemiologia , Nascimento Prematuro/epidemiologia , Adulto , Feminino , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Masculino , Gravidez , Diagnóstico Pré-Natal/estatística & dados numéricos , Estudos Retrospectivos , Fatores de Risco
18.
J Clin Ultrasound ; 48(4): 211-215, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32175628

RESUMO

Research describing fetal arterial structure and function is lacking within the literature. This study's purpose was to measure intima-media thickness (IMT), compliance and distensibility of 24- to 25-week fetuses' abdominal aortas using conventional ultrasonography from 16 diabetic (DM) and 25 non-diabetic mothers (NDM). We found no significant differences in fetal abdominal aorta IMT (P = .68), diameter distensibility (P = .22), or cross-sectional distensibility (P = .23). However, both fetal abdominal aortic diameter compliance (P = .03) and cross-sectional compliance (P = .005) were greater in DM than in NDM (P = .005). This study demonstrates that it is possible and potentially useful to measure fetal abdominal aorta biometrics and biomechanical characteristics.


Assuntos
Aorta Abdominal/diagnóstico por imagem , Espessura Intima-Media Carotídea , Aorta Abdominal/fisiopatologia , Estudos Transversais , Diabetes Mellitus , Estudos de Viabilidade , Feminino , Feto , Idade Gestacional , Humanos , Masculino , Mães , Ultrassonografia
19.
J Pediatr Hematol Oncol ; 42(4): 256-260, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32149865

RESUMO

Although rare in the general pediatric population, the incidence of pericardial effusion is significantly higher in pediatric patients undergoing hematopoietic stem cell transplant (HCT) with a reported incidence of up to 16.9%. The development of pericardial effusion in this setting is associated with higher mortality. Although pericardiocentesis is a relatively safe procedure for treating pericardial effusion, it is invasive, painful, and exposes an immunosuppressed patient to the risks of infection, bleeding, and injury to surrounding structures. Given the procedural risks of pericardiocentesis, systemic steroids are often administered for the treatment of pericardial effusion given their use for pericarditis in the general population. However, the effectiveness of systemic steroids for the treatment of pericardial effusion in the pediatric HCT population has not been confirmed. We studied the role of systemic steroids, administered at the time of initial pericardiocentesis performed for pericardial effusion, in preventing repeat pericardiocentesis. A total of 37 pericardiocenteses after HCT were performed during the study period with 25 patients undergoing first-time pericardiocentesis and 15 of those patients receiving systemic steroids. Eight patients required repeat pericardiocentesis; 5 of 15 (33%) received steroids and 3 of 10 (30%) did not receive steroids. Our data in this small cohort of pediatric HCT patients did not show a significant difference in the need for repeat pericardiocentesis with the use of systemic steroids, initiated within 48 hours of pericardiocentesis.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Derrame Pericárdico , Pericardiocentese , Esteroides/administração & dosagem , Aloenxertos , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Derrame Pericárdico/tratamento farmacológico , Derrame Pericárdico/epidemiologia , Derrame Pericárdico/etiologia , Recidiva
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