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1.
BMJ Case Rep ; 14(1)2021 Jan 11.
Artigo em Inglês | MEDLINE | ID: mdl-33431524

RESUMO

A 34-year-old woman was seen in the emergency department for shortness of breath and chest pain. During a pandemic, it is easy to 'think horses and not zebras', and with a patient presenting with the classic coronavirus symptoms it would have been easy to jump to that as her diagnosis. After a careful history and examination, it became clear that there was another underlying diagnosis. Chest X-ray, echocardiogram and CT scan revealed marked right ventricular dilatation and pulmonary hypertension, alongside a persistent left superior vena cava (PLSVC). Further investigation with cardiac MRI and coronary angiography at a tertiary centre demonstrated that she not only have a PLSVC but also a partial anomalous pulmonary venous drainage and sinus venosus atrial septal defect. This case highlights the importance of considering all differentials and approaching investigations in a logical manner.


Assuntos
/diagnóstico , Dor no Peito/fisiopatologia , Dispneia/fisiopatologia , Comunicação Interatrial/diagnóstico por imagem , Hipertensão Pulmonar/diagnóstico por imagem , Hipertrofia Ventricular Direita/diagnóstico por imagem , Síndrome de Cimitarra/diagnóstico por imagem , Adulto , Cateterismo Cardíaco , Dor no Peito/etiologia , Angiografia por Tomografia Computadorizada , Angiografia Coronária , Diagnóstico Diferencial , Dilatação Patológica/complicações , Dilatação Patológica/diagnóstico por imagem , Dilatação Patológica/fisiopatologia , Dispneia/etiologia , Ecocardiografia , Eletrocardiografia , Feminino , Comunicação Interatrial/complicações , Comunicação Interatrial/fisiopatologia , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/fisiopatologia , Hipertrofia Ventricular Direita/complicações , Hipertrofia Ventricular Direita/fisiopatologia , Imagem por Ressonância Magnética , /fisiopatologia , Síndrome de Cimitarra/complicações , Síndrome de Cimitarra/fisiopatologia , Tomografia Computadorizada por Raios X , Pressão Ventricular
3.
Medicina (Kaunas) ; 56(12)2020 Dec 19.
Artigo em Inglês | MEDLINE | ID: mdl-33352654

RESUMO

The interplay between coronavirus disease 2019 (COVID-19) and pulmonary hypertension (PH) in children is unknown. Adults with PH are at potential risk for severe complications and high mortality due to associated comorbidities. It is difficult to extrapolate the outcomes of COVID-19 in adults to pediatric PH patients. Overall, a small number of COVID-19 cases is reported in patients with preexisting PH. Several factors may be responsible for the low incidence of COVID-19 in children with PH. Pulmonary hypertension is a rare disease, testing is not universal, and patients may have followed more rigorously the Center for Disease Control's guidelines recommended for personal protection with mask-wearing, social distancing, and hand sanitization through ongoing health education. The small number of COVID-19 cases in patients with preexisting PH does not support that PH is protective for COVID-19. However, medications used to treat PH may have some protection against COVID-19. This review discusses the pathophysiology of PH occurring with COVID-19, differences between children and adults with COVID-19, strategies for management of preexisting PH in children during the ongoing pandemic, and its impact within the field of PH.


Assuntos
/complicações , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/fisiopatologia , /epidemiologia , Criança , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/terapia , Incidência , Pandemias
5.
Kyobu Geka ; 73(10): 789-794, 2020 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-33130767

RESUMO

A large population of patients with congenital heart disease with untreated systemic to pulmonary shunts( left to right shunts) will develop pulmonary arterial hypertension( PAH). There are 2 different statuses of an increase in pulmonary arterial pressure. One is high resistance due to high pulmonary blood flow (high flow with high resistance), another one is low pulmonary flow due to high resistance (low flow with high resistance). Chronic large left-to-right shunt induced severe pulmonary vascular disease and pulmonary hypertension. This was then subsequence of low pulmonary blood flow with high pulmonary vascular resistance. We have to avoid this situation and have to do intervention within the pulmonary vascular reactivity has been left. For this reason, preoperative treatment for avoidance of high flow, appropriate timing of interventions and postoperative various managements are important factors as aiming of low pulmonary resistance in this group. Recent advances in PAH-specific drugs have dramatically changed the therapeutic strategy for PAH. A strategy that includes "treatment" with PAH-specific drugs initially and then "repair" by closure of the cardiac defect (i.e. "treat and repair") was devised, and has been attempted, in patients with PAH associated with a cardiac defect.


Assuntos
Cardiopatias Congênitas , Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Humanos , Hipertensão Pulmonar/complicações , Pulmão , Circulação Pulmonar
6.
Medicine (Baltimore) ; 99(43): e22443, 2020 Oct 23.
Artigo em Inglês | MEDLINE | ID: mdl-33120739

RESUMO

The objectives of this study were to describe the prevalence and characteristics of radiographic lesions of the hands, and calcifications of the spine on computer tomography scans (CT-scans), and to investigate the relationships between radiographic and CT-scan abnormalities and clinical features in a population of patients with systemic sclerosis (SSc).Subjects underwent X-ray examination of the hands, and thoracic or thoraco-abdominal and pelvic CT scan or lumbar CT scan in the year. Structural lesions on hand X ray was scored and spinal calcifications were evaluated in the anterior, intracanal and posterior segments. Intra and inter-reliability was tested for radiography and CT- scan. Prognostic factors considered were interstitial pulmonary lesions on the CT scan, pulmonary arterial hypertension (PAH) and death.This study involved 77 SSc patients, 58 (75%) with limited cutaneous SSc (lcSSc) and 19 (25%) with diffuse SSc (dSSc). The prevalences of radiographic lesions of the hand were 28.6% for periarticular calcifications and 26% for calcinosis. On CT scan, 64 (83%) patients exhibited at least 1 calcification. Spine calcifications were depicted in 80.5%, 27.3%, and 35.1% at the anterior, intracanal and posterior segments respectively. Calcifications were mainly localized on thoracic spine. Inter reader reliabilities were good for hands and moderate for spine respectively. Spine calcifications and periarticular calcifications in the hands were associated (P = .012). Calcinosis in the hands was related to PAH (P = .02). Posterior calcification segment and foraminal calcifications were associated with interstitial lung disease (ILD) (P = .029) and death (P = .001).More than 80% of systemic sclerosis patients presented spine calcifications. A significant association between hands and spinal calcifications were confirmed and some localization in the posterior segment considered as a bad prognostic factor.


Assuntos
Calcinose/diagnóstico por imagem , Ossos da Mão/diagnóstico por imagem , Escleroderma Sistêmico/complicações , Doenças da Coluna Vertebral/diagnóstico por imagem , Coluna Vertebral/diagnóstico por imagem , Fatores Etários , Feminino , Humanos , Hipertensão Pulmonar/complicações , Doenças Pulmonares Intersticiais/complicações , Masculino , Pessoa de Meia-Idade , Prognóstico , Radiografia , Reprodutibilidade dos Testes , Estudos Retrospectivos , Escleroderma Sistêmico/mortalidade , Tomografia Computadorizada por Raios X
8.
PLoS One ; 15(9): e0238171, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32925924

RESUMO

Magnetic resonance imaging (MRI) is an emerging tool for diagnosis and treatment monitoring of chronic thromboembolic pulmonary hypertension (CTEPH). The current study aims to identify central pulmonary arterial hemodynamic parameters that reflect clinical, cardiac and pulmonary changes after PEA. 31 CTEPH patients, who underwent PEA and received pre- and postoperative MRI, were analyzed retrospectively. Central pulmonary arterial blood flow, lung perfusion and right heart function data were derived from MRI. Mean pulmonary arterial pressure (mPAP) and 5-month follow-up six-minute walk-distance (6MWD) were assessed. After PEA, mPAP decreased significantly and patients achieved a higher 6MWD. Central pulmonary arterial blood flow velocities, pulmonary blood flow (PBF) and right ventricular function increased significantly. Two-dimensional (2D) phase-contrast (PC) MRI-derived average mean velocity, maximum mean velocity and deceleration volume changes after PEA correlated with changes of 6MWD and right heart ejection fraction (RVEF). Deceleration volume is a novel 2D PC MRI parameter showing further correlation with PBF changes. In conclusion, 2D PC MRI-derived main pulmonary hemodynamic changes reflect changes of RVEF, PBF and 5-month follow-up 6MWD and may be used for future CTEPH patient monitoring after PEA.


Assuntos
Circulação Coronária , Endarterectomia , Hipertensão Pulmonar/fisiopatologia , Hipertensão Pulmonar/cirurgia , Imagem por Ressonância Magnética , Circulação Pulmonar , Trombose/complicações , Idoso , Doença Crônica , Feminino , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Período Perioperatório , Estudos Retrospectivos
9.
Medicine (Baltimore) ; 99(35): e21921, 2020 Aug 28.
Artigo em Inglês | MEDLINE | ID: mdl-32871928

RESUMO

RATIONALE: The anesthetic management of patients with severe pulmonary hypertension is different from that of normal, healthy patients, and regional nerve blocks are commonly used for them. Due to the individual variability of the course, distribution, and branching of the nerves below the inguinal ligament, the supra-inguinal fascia iliaca (SIFI) block has a wider and more stable blocking area. In combination with the sacral plexus block, they can satisfy the needs of surgical anesthesia below the hip. PATIENT CONCERNS: A 46-year-old man with tuberculosis, chronic obstructive pulmonary disease, pulmonary heart disease, World Health Organization (WHO) class III pulmonary hypertension and right heart dysfunction, and American Society of Anesthesiologists physical status class III needed fixation of an intramedullary nail in the left lower extremity. Additionally, he had broken his left lower limb after a recent fall. Both general anesthesia and epidural anesthesia were not appropriate. DIAGNOSES: The patient had a clear history of tuberculosis, computerized tomography scan displayed destructive pneumonophthisis. Furthermore, he had chronic obstructive pulmonary disease and pulmonary heart disease. INTERVENTIONS: An ultrasound-guided SIFI combined with a sacral plexus block was successfully performed for surgical anesthesia and avoided all hemodynamic fluctuations. OUTCOMES: We successfully performed an ultrasound-guided SIFI combined with a sacral plexus block for surgical anesthesia and avoided all hemodynamic fluctuations. LESSONS: Ultrasound-guided suprainguinal fascia iliaca block combined with a sacral plexus block can be suitable for anesthesia for patients with severe circulatory compromise.


Assuntos
Fraturas do Colo Femoral/cirurgia , Fixação Intramedular de Fraturas , Bloqueio Nervoso/métodos , Ultrassonografia de Intervenção/métodos , Pinos Ortopédicos , Fáscia/inervação , Fraturas do Colo Femoral/complicações , Fixação Intramedular de Fraturas/instrumentação , Humanos , Hipertensão Pulmonar/complicações , Plexo Lombossacral , Masculino , Pessoa de Meia-Idade , Doença Pulmonar Obstrutiva Crônica/complicações , Fatores de Risco , Tuberculose Pulmonar/complicações
10.
Med Hypotheses ; 143: 110142, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32759013

RESUMO

BACKGROUND: Pulmonary hypertension is a significant complication for some patients with COVID-19 pneumonia, especially those requiring intensive care. Tachyphylaxis to the current therapy, inhaled nitric oxide (iNO), is also common. In vitro, folic acid directly increases nitric oxide (NO) production and extends its duration of action; effects which could be of benefit in reversing pulmonary hypertension and severe hypoxaemia. Our work has shown that, in the systemic circulation, folic acid in high dose rapidly improves nitric oxide mediated vasodilation, by activating endothelial nitric oxide synthase (eNOS). HYPOTHESIS: A similar effect of high dose folic acid on pulmonary endothelial function would be expected from the same mechanism and would lead to improvement in pulmonary perfusion. We therefore hypothesise that folic acid, 5 mg or greater, is a useful therapeutic option for pulmonary hypertension and/or refractory severe hypoxaemia, in patients with severe COVID-19 associated pneumonia in whom NO therapy is considered, with a very low risk of adverse effects.


Assuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Ácido Fólico/uso terapêutico , Hipertensão Pulmonar/tratamento farmacológico , Óxido Nítrico/metabolismo , Pandemias , Pneumonia Viral/complicações , Administração por Inalação , Animais , Endotélio Vascular/efeitos dos fármacos , Endotélio Vascular/fisiologia , Ativação Enzimática/efeitos dos fármacos , Ácido Fólico/administração & dosagem , Ácido Fólico/farmacologia , Humanos , Hipertensão Pulmonar/complicações , Hipóxia/tratamento farmacológico , Hipóxia/etiologia , Camundongos , Óxido Nítrico/administração & dosagem , Óxido Nítrico/uso terapêutico , Óxido Nítrico Sintase Tipo III/efeitos dos fármacos , Taquifilaxia
11.
J Pediatr ; 225: 246-248, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32610168

RESUMO

With increasing information available about the epidemiology, pathophysiology, and management of patients affected with severe acute respiratory syndrome corona virus-2 infection, patients with Down syndrome, congenital heart disease, airway obstruction, and pulmonary hypertension present a unique challenge. This case series describes 3 patients with Down syndrome and respiratory failure secondary to coronavirus infection.


Assuntos
Infecções por Coronavirus/complicações , Síndrome de Down/complicações , Cardiopatias Congênitas/complicações , Hipertensão Pulmonar/complicações , Pneumonia Viral/complicações , Adulto , Betacoronavirus , Pré-Escolar , Feminino , Humanos , Masculino , Pandemias , Fatores de Risco , Adulto Jovem
12.
J Vis Exp ; (160)2020 06 03.
Artigo em Inglês | MEDLINE | ID: mdl-32568218

RESUMO

Pulmonary Hypertension (PH) is a pathophysiological condition, defined by a mean pulmonary arterial pressure exceeding 25 mm Hg at rest, as assessed by right heart catheterization. A broad spectrum of diseases can lead to PH, differing in their etiology, histopathology, clinical presentation, prognosis, and response to treatment. Despite significant progress in the last years, PH remains an uncured disease. Understanding the underlying mechanisms can pave the way for the development of new therapies. Animal models are important research tools to achieve this goal. Currently, there are several models available for recapitulating PH. This protocol describes a two-hit mouse PH model. The stimuli for PH development are hypoxia and the injection of SU5416, a vascular endothelial growth factor (VEGF) receptor antagonist. Three weeks after initiation of Hypoxia/SU5416, animals develop pulmonary vascular remodeling imitating the histopathological changes observed in human PH (predominantly Group 1). Vascular remodeling in the pulmonary circulation results in the remodeling of the right ventricle (RV). The procedures for measuring RV pressures (using the open chest method), the morphometrical analyses of the RV (by dissecting and weighing both cardiac ventricles) and the histological assessments of the remodeling (both pulmonary by assessing vascular remodeling and cardiac by assessing RV cardiomyocyte hypertrophy and fibrosis) are described in detail. The advantages of this protocol are the possibility of the application both in wild type and in genetically modified mice, the relatively easy and low-cost implementation, and the quick development of the disease of interest (3 weeks). Limitations of this method are that mice do not develop a severe phenotype and PH is reversible upon return to normoxia. Prevention, as well as therapy studies, can easily be implemented in this model, without the necessity of advanced skills (as opposed to surgical rodent models).


Assuntos
Hipertensão Pulmonar/induzido quimicamente , Hipertensão Pulmonar/patologia , Indóis/farmacologia , Pirróis/farmacologia , Animais , Hipóxia Celular/efeitos dos fármacos , Modelos Animais de Doenças , Fibrose , Ventrículos do Coração/efeitos dos fármacos , Ventrículos do Coração/fisiopatologia , Humanos , Concentração de Íons de Hidrogênio , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/fisiopatologia , Hipertrofia Ventricular Direita/complicações , Masculino , Camundongos , Artéria Pulmonar/efeitos dos fármacos , Artéria Pulmonar/fisiopatologia , Circulação Pulmonar/efeitos dos fármacos , Fator A de Crescimento do Endotélio Vascular/metabolismo , Remodelação Vascular/efeitos dos fármacos , Remodelação Ventricular/efeitos dos fármacos
13.
J Cardiothorac Surg ; 15(1): 124, 2020 Jun 04.
Artigo em Inglês | MEDLINE | ID: mdl-32498681

RESUMO

BACKGROUND: Woven coronary artery (WCA) is an extremely rare congenital anomaly in which a part of epicardial coronary artery is divided into thin channels, that twist along the axis of the coronary arteries and then merge again as the main coronary lumen. This anomaly is regarded as a benign condition because the blood flow is normal. Very few cases of WCA have been reported. CASE PRESENTATION: Herein we report a case of a 44-year-old man who was admitted to our hospital due to 20 years of repeated episodes of heart palpitations, 2 years of shortness of breath after activity, and the symptoms were aggravated for 1 month. He had history of inferior myocardial infarction and atrial fibrillation. Color Doppler echocardiography revealed rheumatic heart disease, severe mitral regurgitation, mild-moderate tricuspid regurgitation, moderate pulmonary hypertension. Coronary angiography revealed 60-85% diffuse stenosis in the middle of left anterior descending artery, 60-90% diffuse stenosis in the middle of left circumflex artery, 30-40% diffuse stenosis in the proximal segment of right coronary artery, and WCA anomaly in the middle, and distal segments of right coronary artery. CONCLUSION: The patient successfully underwent prosthetic valve replacement and left anterior descending coronary artery bypass grafting, and had a good recovery after surgery. Further studies are needed to fully understand the disease and determine appropriate treatment options.


Assuntos
Estenose Coronária/diagnóstico por imagem , Anomalias dos Vasos Coronários/diagnóstico por imagem , Insuficiência da Valva Mitral/diagnóstico por imagem , Infarto do Miocárdio/etiologia , Cardiopatia Reumática/diagnóstico por imagem , Adulto , Angiografia Coronária , Ponte de Artéria Coronária , Estenose Coronária/complicações , Estenose Coronária/cirurgia , Anomalias dos Vasos Coronários/complicações , Ecocardiografia Doppler em Cores , Implante de Prótese de Valva Cardíaca , Humanos , Hipertensão Pulmonar/complicações , Masculino , Insuficiência da Valva Mitral/complicações , Insuficiência da Valva Mitral/cirurgia , Cardiopatia Reumática/complicações , Cardiopatia Reumática/cirurgia , Insuficiência da Valva Tricúspide/complicações , Insuficiência da Valva Tricúspide/diagnóstico por imagem
14.
BMC Cardiovasc Disord ; 20(1): 259, 2020 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-32487063

RESUMO

BACKGROUND: Right ventricular (RV) function is a major determinant of outcome in patients with pulmonary hypertension. Cardiac magnetic resonance (CMR) is gold standard to assess RV ejection fraction (RVEFCMR), however this is a crude measure. New CMR measures of RV function beyond RVEFCMR have emerged, such as RV lateral atrio-ventricular plane displacement (AVPDlat), maximum emptying velocity (S'CMR), RV fractional area change (FACCMR) and feature tracking of the RV free wall (FWSCMR). However, it is not fully elucidated if these CMR measures are in parity with the equivalent echocardiography-derived measurements: tricuspid annular plane systolic excursion (TAPSE), S'-wave velocity (S'echo), RV fractional area change (FACecho) and RV free wall strain (FWSecho). The aim of this study was to compare regional RV function parameters derived from CMR to their echocardiographic equivalents in patients with pulmonary hypertension and to RVEFCMR. METHODS: Fifty-five patients (37 women, 62 ± 15 years) evaluated for pulmonary hypertension underwent CMR and echocardiography. AVPDlat, S'CMR, FACCMR and FWSCMR from cine 4-chamber views were compared to corresponding echocardiographic measures and to RVEFCMR delineated in cine short-axis stack. RESULTS: A strong correlation was demonstrated for FAC whereas the remaining measurements showed moderate correlation. The absolute bias for S' was 2.4 ± 3.0 cm/s (relative bias 24.1 ± 28.3%), TAPSE/AVPDlat 5.5 ± 4.6 mm (33.2 ± 25.2%), FWS 4.4 ± 5.8% (20.2 ± 37.5%) and for FAC 5.1 ± 8.4% (18.5 ± 32.5%). In correlation to RVEFCMR, FACCMR and FWSecho correlated strongly, FACecho, AVPDlat, FWSCMR and TAPSE moderately, whereas S' had only a weak correlation. CONCLUSION: This study has demonstrated a moderate to strong correlation of regional CMR measurements to corresponding echocardiographic measures. However, biases and to some extent wide limits of agreement, exist between the modalities. Consequently, the equivalent measures are not interchangeable at least in patients with pulmonary hypertension. The echocardiographic parameter that showed best correlation with RVEFCMR was FWSecho. At present, FACecho and FWSecho as well as RVEFCMR are the preferred methods to assess and follow up RV function in patients with pulmonary hypertension. Future investigations of the CMR right ventricular measures, beyond RVEF, are warranted.


Assuntos
Ecocardiografia , Hipertensão Pulmonar/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Volume Sistólico , Disfunção Ventricular Direita/diagnóstico por imagem , Função Ventricular Direita , Idoso , Feminino , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/fisiopatologia
15.
APMIS ; 128(8): 506-510, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32564430

RESUMO

This case report discusses a patient with nephrotic syndrome, pulmonary hypertension, and repeated episodes of infections. He had a history of intravenous drug abuse. Kidney biopsy revealed the rare finding of numerous foam cells, mainly in glomeruli. The solvent used for the drugs is thought to be responsible for the foam cells. In line with previous reports, we suspect that the pulmonary hypertension is consistent with foam cells in pulmonary capillaries or fat embolism syndrome due to the intravenous administered drugs. Our case demonstrates that the use of intravenous drugs can lead to widely varying symptoms. Globally, the prevalence of substance abuse is increasing. Knowledge about their damaging effects is crucial in both clinical practice and anatomic pathology.


Assuntos
Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/patologia , Infecções/etiologia , Infecções/patologia , Síndrome Nefrótica/etiologia , Síndrome Nefrótica/patologia , Abuso de Substâncias por Via Intravenosa/complicações , Adulto , Biópsia , Células Espumosas/patologia , Humanos , Hipertensão Pulmonar/complicações , Infecções/complicações , Rim/patologia , Masculino , Síndrome Nefrótica/complicações
16.
West Afr J Med ; 37(3): 281-283, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32476123

RESUMO

BACKGROUND: Ventricular Septal Defect (VSD) is the commonest congenital heart disease. Without appropriate treatment, it is associated with significant morbidity and mortality. Surgical repair under cardiopulmonary bypass has been the standard treatment. Results of such treatment is not readily available from the West African sub region. We analysed the outcome of surgical repair of VSDs carried out in this Centre over a 20-year period. PATIENTS AND METHODS: A retrospective study was done for all patients who had surgical repair of VSD from January 1993 to December 2012. RESULTS: There were a total of 207 patients, with 6 and 23 of them operated on in the first and last years of the study respectively. There were 121 (58.5%) males and 86 (41.5%) females. The mean age was 10.0 ± 10.5 (11 months - 55 years), with a median of 7 years. The modal class interval was 0 - 5 years (46.4%). Most of the VSDs were perimembranous 168 (81.1%), followed by outlet VSDs 19 (9.2%), muscular VSDs 11 (5.3%) and inlet VSDs 9 (4.4%). Fifty-four cases (26%) had associated congenital cardiac anomalies that needed concomitant surgical intervention, with the commonest being Pulmonary Stenosis (PS) 21 (10.1%), followed by Patent Ductus Ateriosus (PDA) 10 (4.8%). The complication rate was 6.4% (13 cases), comprising a morbidity of 4.4 % (9 cases) and early mortality of 2.0% (4 cases). The morbidity was due to bleeding requiring re-exploration 2 (1.0%), residual VSD requiring re-do 3 (1.4%), complete heart block requiring permanent pacemaker implantation 2 (1.0%), acute renal failure requiring dialysis 1 (0.5%), sternal wound infection requiring debridement 1 (0.5%). The mortality was due to pulmonary hypertension. CONCLUSION: With a morbidity of 4.4% and early mortality of 2.0%, the outcome of surgical repair of VSDs from this study is good. Children with VSDs must be offered repair as soon as possible to avoid the numerous complications that usually follow untreated VSDs.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interventricular/cirurgia , Septos Cardíacos/cirurgia , Hipertensão Pulmonar/complicações , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Gana/epidemiologia , Comunicação Interventricular/diagnóstico , Comunicação Interventricular/epidemiologia , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
17.
Pediatr Cardiol ; 41(6): 1115-1124, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32388668

RESUMO

Elective closure of atrial septal defect (ASD) is usually recommended during preschool ages. However, ASD may contribute to deteriorating health in the presence of significant comorbidity and, thus, may need earlier closure. There is a lack of clarity regarding the indications for and outcomes after ASD closure in infancy and early childhood. We investigated the benefits and safety of surgical ASD closure in symptomatic patients under 2 years of age. Retrospective chart review was conducted in patients who underwent surgical ASD closure within the first 2 years of life. Of 31 symptomatic ASD patients, 22 had persistent respiratory symptoms, 24 failure to thrive, and 9 pulmonary hypertension. Overall, 26 patients (84.0%) showed clinical improvement after ASD closure, including improved respiratory status (17/22; 77.3%), resumption of normal growth (15/24; 62.5%), and resolution of pulmonary hypertension (7/7; 100%, 2 patients unable to assess postoperatively). Two medically complicated patients died a few months after surgery unrelated to surgical complications. Four out of 8 ventilator-dependent patients were weaned from mechanical ventilation within 1 month after ASD closure. Closure of ASD did not improve those patients with highly advanced lung disease and/or medically complex conditions including underlying genetic abnormalities. Surgical complications were uncommon. Postoperative hospital stay was 4 to 298 days (median 8 days). The majority of our patients demonstrated significant clinical improvement after ASD closure. Early ASD closure is safe and beneficial for symptomatic infants and young children with associated underlying pulmonary abnormalities, especially bronchopulmonary dysplasia.


Assuntos
Comunicação Interatrial/cirurgia , Displasia Broncopulmonar/complicações , Displasia Broncopulmonar/fisiopatologia , Cateterismo Cardíaco/métodos , Pré-Escolar , Feminino , Comunicação Interatrial/complicações , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/fisiopatologia , Lactente , Tempo de Internação , Masculino , Estudos Retrospectivos , Resultado do Tratamento
19.
Presse Med ; 49(2): 104026, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32437844

RESUMO

Idiopathic pulmonary fibrosis (IPF) is characterized by relentlessly progressive lung function impairment that is consistently fatal in the absence of lung transplantation, as no curative pharmacological treatment exists. The pace of progression varies across patients, and acute life-threatening exacerbations occur unpredictably, causing further sharp drops in lung function. Recently introduced antifibrotic agents slow the pace of disease progression and may improve survival but fail to stop the fibrotic process. Moreover, the magnitude and kinetics of the response to these drugs cannot be predicted in the individual patient. These characteristics require that lung transplantation be considered early in the course of the disease. However, given the shortage of donor lungs, lung transplantation must be carefully targeted to those patients most likely to benefit. Current guidelines for lung transplantation listing may need reappraisal in the light of recent treatment advances. Patients with IPF often have multiple comorbidities such as coronary heart disease, frailty, and gastro-oesophageal reflux disease (GERD). Consequently, extensive screening for and effective treatment of concomitant conditions is crucial to appropriate candidate selection and outcome optimisation. A multidisciplinary approach is mandatory. Pulmonologists with expertise in IPF must work closely with lung transplant teams. Careful consideration must be given to preoperative optimisation, surgical technique, and pulmonary rehabilitation to produce the best post-transplantation outcomes.


Assuntos
Fibrose Pulmonar Idiopática/cirurgia , Transplante de Pulmão , Fatores Etários , Anti-Inflamatórios não Esteroides/uso terapêutico , Doenças Cardiovasculares/complicações , Comorbidade , Progressão da Doença , Fragilidade/complicações , Refluxo Gastroesofágico/complicações , Humanos , Hipertensão Pulmonar/complicações , Pneumonias Intersticiais Idiopáticas/complicações , Indóis/uso terapêutico , Neoplasias Pulmonares/complicações , Prognóstico , Piridonas/uso terapêutico , Encurtamento do Telômero
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