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1.
Tex Heart Inst J ; 46(3): 225-228, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31708710

RESUMO

Anomalous origin of the right coronary artery from the pulmonary artery, a rare congenital cardiac defect, is typically not diagnosed during infancy. On the other hand, Turner syndrome is usually diagnosed early, and it is classically associated with bicuspid aortic valve and aortic coarctation. Individuals with Turner syndrome are also at increased risk for coronary artery anomalies. We present a case of anomalous right coronary artery from the pulmonary artery in a week-old neonate who also had Turner syndrome, patent ductus arteriosus, transverse aortic arch hypoplasia, and impaired ventricular function. Prostaglandin therapy through the ductus increased the patient's myocardial perfusion. Four months after corrective surgery, she was doing well. We discuss the reperfusion phenomenon in our patient's case, as well as other considerations in this combination of congenital defects.


Assuntos
Anormalidades Múltiplas , Aorta Torácica/anormalidades , Anomalias dos Vasos Coronários/diagnóstico , Vasos Coronários/diagnóstico por imagem , Artéria Pulmonar/anormalidades , Síndrome de Turner/diagnóstico , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Implante de Prótese Vascular/métodos , Cateterismo Cardíaco , Angiografia por Tomografia Computadorizada , Anomalias dos Vasos Coronários/cirurgia , Vasos Coronários/cirurgia , Ecocardiografia , Feminino , Fluoroscopia , Humanos , Recém-Nascido , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia
3.
Artif Organs ; 43(11): 1085-1091, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31188477

RESUMO

The objective of this study was to describe a single-center experience with neonatal and pediatric extracorporeal life support (ECLS) and compare patient-related outcomes with those of the Extracorporeal Life Support Organization (ELSO) Registry. A retrospective review of subject characteristics, outcomes, and complications of patients who received the ECLS at Penn State Health Children's Hospital (PSHCH) from 2000 to 2016 was performed. Fisher's exact test was used to compare the PSHCH outcomes and complications to the ELSO Registry report. Data from 118 patients were included. Survival to discontinuation of the ECLS was 70.3% and 65.2% to discharge/transfer. Following circuitry equipment changes, the survival to discharge/transfer improved for both neonatal (<29 days) and pediatric (29 days to <18 years) patients. The most common complications associated with ECLS were clinical seizures, intracranial hemorrhage, and culture-proven infection. ECLS for pulmonary support appeared to be associated with a higher risk of circuit thrombus and cannula problems. When compared to the ELSO Registry, low volume ECLS centers, like our institution, can have outcomes that are no different or statistically better as noted with neonatal and pediatric cardiac patients. Pediatric patients requiring pulmonary support appeared to experience more mechanical complications during ECLS suggesting the need for ongoing technological improvement.

6.
World J Pediatr Congenit Heart Surg ; 9(5): 550-556, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-30157737

RESUMO

BACKGROUND: In patients with trisomy 18, congenital heart surgery is controversial due to anticipated poor patient outcome. Data are lacking regarding clinical outcomes and family opinions about care received. METHODS: A retrospective chart review of patients with trisomy 18 and congenital heart disease from 2005 to 2017 was performed. Patients were grouped into those receiving cardiac intervention (surgery or cardiac catheterization) versus medical management. A telephone survey was used to assess completeness of family counseling provided prior to treatment selection and parental opinions on the care received. RESULTS: Seventeen infants were assessed. In the medical management group (n = 7), there were five deaths at a median age of 1.5 months (range: 1.2-4.1 months) and two survivors aged 29 and 44 months at latest follow-up. In the intervention group (n = 10), cardiac surgery was performed in nine patients at a median age of 4.3 months (0.2-23.4 months) and weight of 3.2 kg (1.5-12.2 kg); catheter intervention was performed in one patient at one week of age. At latest follow-up, seven intervention patients are alive at a median age of 50 months (5-91 months). Survey respondents (n = 12) unanimously stated that their child's quality of life was improved by their specific treatment strategy, that the experience of the parents was enhanced, and that they would choose the same treatment course again. CONCLUSIONS: Surgical repair may be associated with favorable early outcomes and may be judiciously offered in selected circumstances. In this limited experience, parental perceptions were positive regarding the quality of care and overall experience independent of the chosen treatment strategy or eventual outcome.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Pais/psicologia , Síndrome da Trissomía do Cromossomo 18/cirurgia , Feminino , Humanos , Lactente , Masculino , Qualidade de Vida , Estudos Retrospectivos , Inquéritos e Questionários , Resultado do Tratamento
8.
Artif Organs ; 42(9): 918-921, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29660857

RESUMO

The objective of this study was to compare the effects of nonpulsatile and ECG-synchronized pulsatile extracorporeal life support on coronary and carotid blood flow velocities using transthoracic echocardiography and vascular ultrasound, respectively. Nine adult swine were randomly separated into nonpulsatile (NP, n = 5) and pulsatile (P, N = 4) groups and placed on ECLS for 24 h using an i-cor ECLS system. Noninvasive transthoracic images of the left and right coronary artery and the left carotid artery were acquired at the pre-ECLS (baseline), 30 min, 3, 6, 9, 12, and 24 h on-ECLS stages. The mean diastolic velocity of the left and right coronary arteries in the NP group significantly decreased after 24 h on ECLS compared to the baseline and 30 min ECLS stages (P < 0.05). There was no statistical difference in the mean diastolic velocity of the coronary arteries in the P group at 30 min, 3-, 6-, 9-, 12-, and 24-h ECLS compared to baseline. The P group showed a smaller decrease in the mean diastolic velocity of coronary arteries between the 30-min ECLS and 3-, 6-, 9-, 13-, 24-h ECLS stages compared to the NP group. The diastolic velocity of the left carotid artery in the NP group significantly decreased during 24-h ECLS compared to the P group (P < 0.05). An ECG-synchronized pulsatile ECLS system appeared to maintain coronary and carotid artery diastolic velocities better than conventional nonpulsatile ECLS. Further investigation of the perfusion modes during ECLS is warranted.


Assuntos
Velocidade do Fluxo Sanguíneo/fisiologia , Pressão Sanguínea/fisiologia , Artérias Carótidas/fisiologia , Vasos Coronários/fisiologia , Oxigenação por Membrana Extracorpórea/métodos , Fluxo Sanguíneo Regional/fisiologia , Animais , Hemodinâmica/fisiologia , Modelos Animais , Fluxo Pulsátil/fisiologia , Suínos , Resistência Vascular/fisiologia
9.
Interact Cardiovasc Thorac Surg ; 25(6): 985-986, 2017 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-29049532

RESUMO

Chronic pulmonary insufficiency following transannular patch repair of tetralogy of Fallot may mandate restoration of a competent pulmonary valve. Pulmonary valve leaflets that are preserved at initial surgery may grow and develop normal morphology and subsequent valve repair may be possible. We reviewed our experience with native pulmonary valve restitution following transannular patch repair (2001-15). The cohort included 9 patients with a median age of 18.7 (range 10.6-31.3) years. Operative technique involved reapproximation of the anterior commissure of the pulmonary valve. Median length of stay was 3 days, and there were no deaths. At median follow-up of 2.0 (0.4-13.5) years, pulmonary insufficiency was graded as ≤mild (n = 4), mild-moderate or moderate (n = 4) and moderate-severe (n = 1); pulmonary stenosis was ≤mild (n = 8) and moderate (n = 1), with median peak gradient of 21 (16-64) mmHg. No patient required reintervention. At reoperation to treat pulmonary insufficiency in repaired TOF, if residual leaflets are found with favourable anatomy, restitution of the native valve should be considered. This valve-preserving technique avoids the certain failure of a bioprosthesis and is associated with favourable early outcomes. The viability of this option may influence surgeons to leave the pulmonary leaflets in situ at the time of initial repair.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Previsões , Implante de Prótese de Valva Cardíaca/métodos , Complicações Pós-Operatórias/cirurgia , Insuficiência da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Adolescente , Adulto , Criança , Feminino , Seguimentos , Humanos , Masculino , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/etiologia , Insuficiência da Valva Pulmonar/diagnóstico , Insuficiência da Valva Pulmonar/etiologia , Reoperação , Resultado do Tratamento , Adulto Jovem
12.
Pediatr Emerg Care ; 32(8): 536-7, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27018525

RESUMO

Nail gun injuries primarily occur in the extremities of adult males as a consequence of accidental occupational trauma. Such injury involving the thorax is much less common, and penetrating cardiac injury secondary to pneumatic nail gun discharge is rare. Although potentially lethal, most cases with cardiac trauma are survivable with expedient surgical intervention. Despite improvements in engineered safety mechanisms, the incidence of nail gun injuries has risen as use of the devices has increased. The widespread availability of these tools to nonprofessional consumers exposes a broader population to the potential hazards associated with these devices. We describe the presentation and successful management of the first reported case of penetrating cardiac nail gun injury in a young child.


Assuntos
Materiais de Construção/efeitos adversos , Traumatismos Cardíacos/etiologia , Ventrículos do Coração/lesões , Ferimentos Penetrantes/etiologia , Pré-Escolar , Feminino , Armas de Fogo , Humanos , Tomografia Computadorizada por Raios X
16.
Artif Organs ; 39(7): E90-E101, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25866125

RESUMO

The primary objective of this study was to evaluate a novel electrocardiogram (ECG)-synchronized pulsatile extracorporeal life support (ECLS) system for adult partial mechanical circulatory support for adequate quality of pulsatility and enhanced hemodynamic energy generation in an in vivo animal model. The secondary aim was to assess end-organ protection during nonpulsatile versus synchronized pulsatile flow mode. Ten adult swine were randomly divided into a nonpulsatile group (NP, n = 5) and pulsatile group (P, n = 5), and placed on ECLS for 24 h using an i-cor system consisting of an i-cor diagonal pump, an iLA membrane ventilator, an 18 Fr femoral arterial cannula and a 23/25 Fr femoral venous cannula. Trials were conducted at a flow rate of 2.5 L/min using nonpulsatile or pulsatile mode (with assist ratio 1:1). Real-time pressure and flow data were recorded using a custom-based data acquisition system. To the best of our knowledge, the oxygenator and circuit pressure drops were the lowest for any available system in both groups. The ECG-synchronized i-cor ECLS system was able to trigger pulsatile flow in the porcine model. After 24-h ECLS, energy equivalent pressure, surplus hemodynamic energy, and total hemodynamic energy at preoxygenator and prearterial cannula sites were significantly higher in the P group than those in the NP group (P < 0.05). Urine output was higher in P versus NP (3379 ± 443 mL vs. NP, 2598 ± 1012 mL), and the P group seemed to require less inotropic support, but both did not reach statistical significances (P > 0.05). The novel i-cor system performed well in the nonpulsatile and ECG-synchronized pulsatile mode in an adult animal ECLS model. The iLA membrane oxygenator had an extremely lower transmembrane pressure gradient and excellent gas exchange capability. Our findings suggest that ECG-triggered pulsatile ECLS provides superior end-organ protection with improved renal function and systemic vascular tone.


Assuntos
Oxigenação por Membrana Extracorpórea , Hemodinâmica , Sistemas de Manutenção da Vida , Animais , Eletrocardiografia , Desenho de Equipamento , Oxigenação por Membrana Extracorpórea/instrumentação , Feminino , Sistemas de Manutenção da Vida/instrumentação , Modelos Animais , Polienos/química , Fluxo Pulsátil , Suínos
17.
Ann Thorac Surg ; 99(5): 1692-8; discussion 1698-9, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25818568

RESUMO

BACKGROUND: Bovine pericardial valves are often used for pulmonary valve replacement (PVR) in patients with previously repaired congenital heart disease. Attention has recently focused on the safety of the Mitroflow (Sorin Group USA, Arvada, CO) bovine pericardial valve after a national alert describing several cases of sudden valve failure in young patients. In response, we reviewed our experience using the Mitroflow bioprosthesis for PVR. METHODS: Medical records were reviewed for all patients who underwent PVR using a Mitroflow valve at our center (2008-2013). RESULTS: The cohort included 84 patients with a median age of 18.3 years (range, 0.8-62.1 years) and weight of 48.4 kg (range, 5.7-167.8 kg). Indications for surgical intervention included native outflow tract insufficiency (59 patients), valved conduit failure (20 patients), and isolated prosthetic valve failure (5 patients). Median length of stay was 3 days (range, 2-13 days). There were no hospital deaths. Median follow-up was 2.4 years (range, 0.2-5.6 years). Pulmonary valve insufficiency and peak gradient increased with time. At latest follow-up, freedom from insufficiency greater than or equal to a moderate degree was 83%, and freedom from a peak gradient greater than or equal to 50 mm Hg was 92%. Reintervention was required in 4 patients. One patient experienced endocarditis and underwent surgical PVR (0.6 years after initial intervention). Three patients underwent transcatheter treatment for valve stenosis including PVR (at 4.2 and 4.4 years in 2 patients) and balloon valvuloplasty (at 5.2 years in the third patient). Kaplan-Meier freedom from reintervention at 5 years was 81%. CONCLUSIONS: Early outcomes using the Mitroflow bovine pericardial valve for PVR in children and adults with repaired congenital heart disease appear acceptable and similar to reported outcomes for other tissue valve options. Valve failure from premature structural deterioration was not observed.


Assuntos
Bioprótese , Cardiopatias Congênitas/complicações , Doenças das Valvas Cardíacas/cirurgia , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Valva Pulmonar , Adolescente , Adulto , Animais , Bovinos , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/cirurgia , Doenças das Valvas Cardíacas/complicações , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Pericárdio , Desenho de Prótese , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
18.
Artif Organs ; 39(1): 28-33, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25626577

RESUMO

Apolipoprotein E (apoE) may play a critical role in modulating the response to neurological injury after cardiopulmonary bypass (CPB) in children. Plasma samples were collected from 38 pediatric patients. Half of the patients received nonpulsatile flow and the other half underwent pulsatile flow during CPB. Plasma samples were collected at three time points: at baseline prior to incision (T1), 1 h after CPB (T2), and 24 h after CPB (T3). The study included 38 pediatric patients undergoing heart surgery (mean age 2.5 ± 2.1 years). Baseline apoE levels were low (<30 µg/mL) in 21 patients (55%). ApoE levels were significantly decreased at 1 h after CPB compared with baseline (22 ± 14 vs. 34 ± 18 µg/mL, P = 0.001). At 24 h after CPB, apoE levels were significantly increased compared with baseline (47 ± 25 vs. 34 ± 18 µg/mL, P = 0.002). Pulsatile mode was associated with lower apoE levels at 24 h after CPB compared with nonpulsatile mode (38 ± 14 vs. 57 ± 29 µg/mL, P = 0.018). ApoE levels correlated negatively with pump time (r = -0.525, P = 0.021) and cross-clamp time (r = -0.464, P = 0.045) at 24 h following CPB for the nonpulsatile group but not for the pulsatile group. In this cohort of young children with congenital heart disease, baseline apoE levels were low in the majority of patients prior to surgery. ApoE levels decreased further at 1 h after CPB, and then significantly increased by 24 h. The mode of perfusion and the duration of pump time and clamp time influence the apoE levels after CPB. An improved understanding of these mechanisms may translate into the development of new techniques to improve the clinical outcomes after pediatric CPB.


Assuntos
Apolipoproteínas E/sangue , Procedimentos Cirúrgicos Cardíacos/métodos , Ponte Cardiopulmonar/métodos , Cardiopatias Congênitas/cirurgia , Fluxo Pulsátil , Biomarcadores/sangue , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Ponte Cardiopulmonar/efeitos adversos , Distribuição de Qui-Quadrado , Pré-Escolar , Estudos de Coortes , Feminino , Cardiopatias Congênitas/sangue , Humanos , Lactente , Masculino , Perfusão , Cuidados Pós-Operatórios/métodos , Cuidados Pré-Operatórios/métodos , Prognóstico , Estudos Prospectivos , Medição de Risco , Sensibilidade e Especificidade , Resultado do Tratamento
19.
Artif Organs ; 39(1): 67-76, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25626581

RESUMO

Arrhythmias are a frequent complication during extracorporeal life support (ECLS). A new ECLS system can provide pulsatile flow synchronized to the patient's intrinsic cardiac cycle based upon the R wave of the electrocardiogram (ECG). It is unclear how the occurrence of arrhythmias may alter the hemodynamic performance of the system. This in vitro study evaluated the effect of simulated arrhythmias on hemodynamics during R wave-triggered pulsatile ECLS. The ECLS circuit with an i-cor diagonal pump and iLA membrane ventilator was primed with whole blood at room temperature. Flow and pressure data were collected at 2.5 and 4 L/min for each condition using a customized data acquisition system. Pulsatile ECLS flow was R wave synchronized to an ECG simulator using 1:1, 1:2, and 1:3 assist ratios. Conditions tested included sinus rhythm at 45 and 90 bpm, supraventricular tachycardia (SVT), ventricular tachycardia (VT), and irregular rhythms such as ventricular fibrillation. Pulsatile mode was successfully triggered by ECG signals of normal sinus rhythm, SVT, VT, atrial fibrillation, atrial flutter, and ventricular bigeminy with assist ratios 1:1, 1:2, and 1:3. Regular rhythm at 90 bpm generated the best surplus hemodynamic energy (SHE). For SVT and VT, an assist ratio of 1:2 resulted in maximum pulsatile flow waveforms with optimal SHE at 2.5 L/min flow rate. At 4 L/min, SHE declined and the pressure drop increased independent of arrhythmia condition. Irregular rhythms still produced adequate pulsatile wave forms at lower pulsatile frequency. This study demonstrated the feasibility of generating pulsatile ECLS flow with the novel ECG-synchronized i-cor system during various simulated rhythms. The optimal rate for pulsatile flow was 90 bpm. During irregular rhythms, the lower pulsatile frequency was the more reliable synchronization mode for generating pulsatile flow.


Assuntos
Arritmias Cardíacas/etiologia , Oxigenação por Membrana Extracorpórea/efeitos adversos , Hemodinâmica/fisiologia , Fluxo Pulsátil/fisiologia , Arritmias Cardíacas/diagnóstico , Eletrocardiografia/métodos , Oxigenação por Membrana Extracorpórea/instrumentação , Coração Auxiliar/efeitos adversos , Humanos , Técnicas In Vitro , Sistemas de Manutenção da Vida/instrumentação , Modelos Cardiovasculares , Projetos Piloto , Fatores de Tempo
20.
Artif Organs ; 39(1): 59-66, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25586773

RESUMO

The objective of this study is to investigate the impact of every component of extracorporeal life support (ECLS) circuit on hemodynamic energy transmission in terms of energy equivalent pressure (EEP), total hemodynamic energy (THE), and surplus hemodynamic energy (SHE) under nonpulsatile and pulsatile modes in a novel ECLS system. The ECLS circuit consisted of i-cor diagonal pump and console (Xenios AG, Heilbronn, Germany), an iLA membrane ventilator (Xenios AG), an 18 Fr femoral arterial cannula, a 23/25 Fr femoral venous cannula, and 3/8-in ID arterial and venous tubing. The circuit was primed with lactated Ringer's solution and human whole blood (hematocrit 33%). All trials were conducted under room temperature at the flow rates of 1-4 L/min (1 L/min increments). The pulsatile flow settings were set at pulsatile frequency of 75 beats per minute and differential speed values of 1000-4000 rpm (1000 rpm increments). Flow and pressure data were collected using a custom-based data acquisition system. EEP was significantly higher than mean arterial pressure in all experimental conditions under pulsatile flow (P < 0.01). THE was also increased under pulsatile flow compared with the nonpulsatile flow (P < 0.01). Under pulsatile flow conditions, SHE was significantly higher and increased differential rpm resulted in significantly higher SHE (P < 0.01). There was no SHE generated under nonpulsatile flow. Energy loss depending on the circuit components was almost similar in both perfusion modes at all different flow rates. The pressure drops across the oxygenator were 3.8-24.9 mm Hg, and the pressure drops across the arterial cannula were 19.3-172.6 mm Hg at the flow rates of 1-4 L/min. Depending on the pulsatility setting, i-cor ECLS system generates physiological quality pulsatile flow without increasing the mean circuit pressure. The iLA membrane ventilator is a low-resistance oxygenator, and allows more hemodynamic energy to be delivered to the patient under pulsatile mode. The 18 Fr femoral arterial cannula has acceptable pressure drops under nonpulsatile and pulsatile modes. Further in vivo studies are warranted to confirm these results.


Assuntos
Oxigenação por Membrana Extracorpórea/instrumentação , Hemodinâmica/fisiologia , Sistemas de Manutenção da Vida , Modelos Cardiovasculares , Desenho de Equipamento , Segurança de Equipamentos , Coração Auxiliar , Humanos , Técnicas In Vitro , Oxigenadores , Perfusão , Fluxo Pulsátil/fisiologia
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