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1.
Medicine (Baltimore) ; 98(41): e17333, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31593082

RESUMO

Patients with structural heart disease (SHD) are more difficult to ablate than those with a structurally healthy heart. The reason may be technical problems. We compared periprocedural data in unselected patients (including SHD group) recruited for zero-fluoroscopy catheter ablation (ZF-CA) of supraventricular arrhythmias (SVTs).Consecutive adult patients with atrioventricular nodal reentry tachycardia (AVNRT), accessory pathways (AP), atrial flutter (AFL), and atrial tachycardia (AT) were recruited. A 3-dimensional electroanatomical mapping system (Ensite Velocity, NavX, St Jude Medical, Lake Bluff, Illinois) was used to create electroanatomical maps and navigate catheters. Fluoroscopy was used on the decision of the first operator after 5 minutes of unresolved problems.Of the 1280 patients ablated with the intention to be treated with ZF approach, 174 (13.6%) patients with SHD (age: 58.2 ±â€Š13.6; AVNRT: 23.9%; AP: 8.5%; AFL: 61.4%; and AT: 6.2%) were recruited. These patients were compared with the 1106 patients with nonstructural heart disease (NSHD) (age: 51.4 ±â€Š16.4; AVNRT: 58.0%; AP: 17.6%; AFL: 20.7%; and AT: 3.7% P ≤ .001). Procedural time (49.9 ±â€Š24.6 vs 49.1 ±â€Š23.9 minutes, P = .55) and number of applications were similar between groups (P = 0.08). The rate of conversion from ZF-CA to fluoroscopy was slightly higher in SHD as compared to NSHD (13.2% vs 7.8%, P = .02) while the total time of fluoroscopy and radiation doses were comparable in the group of SHD and NSHD (P = .55; P = .48).ZF-CA is feasible and safe in majority of patients with SHD and should be incorporated into a standard approach for SHD; however, the procedure requires sufficient experience.


Assuntos
Arritmias Cardíacas/cirurgia , Ablação por Cateter/métodos , Fluoroscopia/métodos , Cardiopatias Congênitas/cirurgia , Taquicardia Supraventricular/cirurgia , Adulto , Idoso , Arritmias Cardíacas/congênito , Estudos de Viabilidade , Feminino , Cardiopatias Congênitas/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Taquicardia Supraventricular/congênito , Resultado do Tratamento
2.
Medicine (Baltimore) ; 98(42): e17413, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31626096

RESUMO

INTRODUCTION: VACTERL association is an acronym that includes vertebral anomalies (V), anal atresia (A), cardiac defects (C), tracheoesophageal fistula (TEF) or esophageal atresia (EA), renal anomalies (R), and limb defects (L). Airway anomalies have rarely been reported with VACTERL association. PATIENT CONCERNS: A 10-month-old boy who had been diagnosed with anal atresia and received surgical corrections soon after birth consulted our institution by complaining repeated cough and fever. DIAGNOSIS: Diagnosis of VACTERL association was finally made. Bronchoscopy and chest CT with computed tomography angiography confirmed multiple airway abnormalities including bridging bronchus, airway malacia, and complete tracheal rings. INTERVENTIONS: Supplemental oxygen was provided and antibiotics was initiated. OUTCOMES: The patient resolved gradually and was discharged 10 days later. The follow-up showed the patient has remained well just with mild psychomotor retardation. CONCLUSION: Multiple airway anomalies may be seen in VACTERL association. It is worthwhile to make special note for evaluating the tracheobronchial pulmonary system by chest CT and bronchoscopy, especially patients presenting with breathing anomalies.


Assuntos
Canal Anal/anormalidades , Esôfago/anormalidades , Cardiopatias Congênitas/diagnóstico , Rim/anormalidades , Deformidades Congênitas dos Membros/diagnóstico , Coluna Vertebral/anormalidades , Traqueia/anormalidades , Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/etiologia , Manuseio das Vias Aéreas/métodos , Broncoscopia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/terapia , Humanos , Lactente , Deformidades Congênitas dos Membros/complicações , Deformidades Congênitas dos Membros/terapia , Masculino , Oxigenoterapia , Tórax/anormalidades , Tórax/diagnóstico por imagem , Tibet , Tomografia Computadorizada por Raios X
3.
Medicine (Baltimore) ; 98(40): e17449, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31577770

RESUMO

BACKGROUND: Pulmonary artery sling (PAS) is rare, often with tracheal stenosis. And the postoperative mortality is high. For now, there is no consensus on the tracheoplasty for the patients with PAS and tracheal stenosis. METHODS: Studies involving surgical repair of PAS and tracheal stenosis with and without tracheoplasty were identified by searching the PubMed, EMBASE, and the Cochrane Library databases until June 5, 2019. The assessed variables included ventilation time, early and late mortality, and symptom at follow-up. A random-effect/fixed-effect model was used to summarize the estimates of the mean difference (MD)/risk ratio (RR) with 95% confidence interval (CI). RESULTS: This study will be submitted to a peer-reviewed journal for publication. CONCLUSION: This study will assess the safety and efficacy of tracheoplasty for patients with PAS and tracheal stenosis, and provide more evidence-based guidance in clinical practice. PROSPERO REGISTRATION NUMBER: CRD42019139788.


Assuntos
Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Artéria Pulmonar/anormalidades , Procedimentos Cirúrgicos Reconstrutivos , Estenose Traqueal/complicações , Estenose Traqueal/cirurgia , Humanos
5.
Zhonghua Er Ke Za Zhi ; 57(9): 705-709, 2019 Sep 02.
Artigo em Chinês | MEDLINE | ID: mdl-31530357

RESUMO

Objective: To characterize the clinical features and outcomes of scimitar syndrome (SS) to aid the understanding of this syndrome. Methods: This retrospective study included 6 children who were diagnosed with SS at the pediatric cardiovascular center of Beijing Anzhen Hospital from January 2012 to September 2018. SS was diagnosed by echocardiography and confirmed by cardiac computed tomography angiography(CTA) or surgery. All data were collected to analyze the clinical and imaging characteristics and prognosis. Results: Among the 6 SS children (aged 2 months to 15 years; 5 males) weighed 5.6-17.1 kg. Three cases were infant type, the clinical manifestations were recurrent respiratory tract infection with growth retardation, including 2 cases with severe pulmonary hypertension, while 3 cases with adult type, were asymptomatic. Cardiac CTA imaging showed that the right single or all pulmonary veins descended through the diaphragm and converged into the inferior vena cava. One case was isolated infracardiac partial anomalous pulmonary venous connection (PAPVC) without other malformations. The remaining 5 cases complicated with atrial septal defect, different vascular and trachea malformations as well as spinal malformations. Vascular malformations included pulmonary veins stenosis, abnormal origin of pulmonary artery branches, collateral branches of systemic artery supplying local lung tissue, and persistent left superior vena cava. The treatment varied according to the specific location of anomalous pulmonary venous connection, the degree of pulmonary hypertension and the severity of clinical symptoms. Four cases underwent one-stage radical surgery, one case accepted intervention to occlude the collateral artery which was supplying the right lower lung and received stage Ⅱ radical surgery half a year later, and the remaining one case died from pulmonary hypertension crisis preoperation. Conclusions: Isolated SS can easily miss diagnosis due to mild clinical symptoms. Patients with complicated malformations can benefit from combination therapy. SS associated with severe pulmonary hypertension can lead to early death. Therefore, early diagnosis and appropriate treatment can improve the prognosis of patients.


Assuntos
Anormalidades Múltiplas/diagnóstico , Síndrome de Cimitarra/diagnóstico , Adolescente , Criança , Pré-Escolar , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico , Comunicação Interatrial/complicações , Humanos , Lactente , Masculino , Artéria Pulmonar/anormalidades , Veias Pulmonares/anormalidades , Infecções Respiratórias/complicações , Estudos Retrospectivos , Síndrome de Cimitarra/etiologia , Síndrome de Cimitarra/mortalidade , Síndrome de Cimitarra/terapia
6.
Rev Port Cir Cardiotorac Vasc ; 26(2): 147-149, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31476817

RESUMO

We report the case of a 44 year-old patient with complex ACHD, admitted with acute decompensated heart failure (ADHF) in hemodynamic profile B. He had a single ventricle with pulmonary atresia, previously submitted to three modified Blalock-Taussig shunts (mBTs) at the age of 2, 12 and 19 years old. Despite conventional treatment with diuretics, ß-blockers (BB) and isosorbide dinitrate the patient progressed to profile C and the transthoracic echocardiogram disclosed a reduced systolic function. Likewise, levosimendan was commenced and an appropriate decongestion and a marked reduction in the NT-proBNP were seen. Treatment with angiotensin-converting-enzyme inhibitor, BB, ivabradine and mineralocorticoid receptor was optimized. The patient was discharged home after 26 days in NYHA class III and referred for heart transplant after right heart catheterization. To our knowledge, this is the first report of successful levosimendan's use in ADHF in a mBTs long-term survivor.


Assuntos
Fármacos Cardiovasculares/uso terapêutico , Cardiopatias Congênitas/cirurgia , Insuficiência Cardíaca/tratamento farmacológico , Ventrículos do Coração/anormalidades , Simendana/uso terapêutico , Adulto , Procedimento de Blalock-Taussig , Cardiopatias Congênitas/complicações , Insuficiência Cardíaca/etiologia , Ventrículos do Coração/cirurgia , Humanos , Masculino , Artéria Pulmonar/anormalidades , Artéria Pulmonar/cirurgia , Resultado do Tratamento
7.
S D Med ; 72(6): 272-273, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31461233

RESUMO

Quadricuspid aortic valve is a rare cause of symptomatic aortic regurgitation. It tends to present earlier than degenerative native valve aortic regurgitation but with similar symptoms. The condition can occur in isolation or in association with other congenital cardiac abnormalities. Surgical intervention before the development of left ventricular failure is critical to improve long term survival. We describe a case of severe aortic regurgitation due to isolated quadricuspid valve morphology that was treated surgically.


Assuntos
Insuficiência da Valva Aórtica , Valva Aórtica/patologia , Cardiopatias Congênitas , Insuficiência Cardíaca , Insuficiência da Valva Aórtica/diagnóstico , Insuficiência da Valva Aórtica/etiologia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico , Humanos
8.
Med Clin North Am ; 103(5): 945-956, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31378336

RESUMO

Cardiac defects are the most common congenital defects, accounting for approximately 9 per 1000 births. Patients with structural heart disease related to congenital diseases are prone to develop intrinsic rhythm abnormalities as a result of altered physiology. In addition, they are at an increased risk of developing acquired arrhythmias secondary to the nature of surgical interventions done to improve physiologic function in the setting of these defects. Arrhythmia management and risk stratification pose particularly complex challenges to clinicians managing this population.


Assuntos
Fibrilação Atrial/diagnóstico , Fibrilação Atrial/terapia , Cardiopatias Congênitas/epidemiologia , Fibrilação Atrial/etiologia , Ablação por Cateter , Gerenciamento Clínico , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Humanos , Marca-Passo Artificial
9.
Methodist Debakey Cardiovasc J ; 15(2): 105-110, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384373

RESUMO

Patients with a functionally univentricular heart who have had an atriopulmonary Fontan are at risk for atrial dilatation, atrial arrhythmias, and progressive circulatory failure. Between 1994 and 2018, we performed 149 Fontan conversions with arrhythmia surgery and epicardial pacemaker placement at Ann & Robert H. Lurie Children's Hospital of Chicago. This operation converts the atriopulmonary Fontan to an extracardiac Fontan that improves hemodynamics and controls the atrial arrhythmias. Operative mortality during that time was 2%, and freedom from death or heart transplant at 10 years is 84%. For properly selected patients, Fontan conversion improves both survival and quality of life. Patients with an atriopulmonary Fontan constitute an eroding population, as they face many comorbidities and have a decreased life expectancy without treatment; therefore, all patients with an atriopulmonary Fontan should be evaluated for this procedure.


Assuntos
Arritmias Cardíacas/cirurgia , Cardiomegalia/cirurgia , Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Adolescente , Adulto , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/mortalidade , Arritmias Cardíacas/fisiopatologia , Cardiomegalia/etiologia , Cardiomegalia/mortalidade , Cardiomegalia/fisiopatologia , Criança , Pré-Escolar , Tomada de Decisão Clínica , Feminino , Técnica de Fontan/efeitos adversos , Técnica de Fontan/mortalidade , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Intervalo Livre de Progressão , Reoperação , Medição de Risco , Fatores de Risco , Fatores de Tempo , Adulto Jovem
10.
Methodist Debakey Cardiovasc J ; 15(2): 145-148, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384378

RESUMO

Steady advances in the diagnosis and management of congenital heart disease over the last few decades has resulted in a growing population of adults with congenital heart disease (ACHD). Consequently, there has been a parallel increase in the number of ACHD patients plagued with end-stage heart failure. Even so, the transplantation rate for these patients has remained low, at about 3% of all adult heart transplants. This review discusses the scope of transplantation for ACHD, including indications and contraindications, specific challenges and nuances, and post-transplant outcomes.


Assuntos
Cardiopatias Congênitas/cirurgia , Insuficiência Cardíaca/cirurgia , Transplante de Coração , Adulto , Fatores Etários , Tomada de Decisão Clínica , Progressão da Doença , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/fisiopatologia , Transplante de Coração/efeitos adversos , Transplante de Coração/mortalidade , Humanos , Seleção de Pacientes , Medição de Risco , Fatores de Risco , Resultado do Tratamento , Listas de Espera
11.
Methodist Debakey Cardiovasc J ; 15(2): 158-159, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384382

RESUMO

This column is supplied by Benjamin J. Lee, MD, MAS, an assistant professor of clinical medicine at both the Houston Methodist Institute for Academic Medicine and Weill Cornell Medical College. After earning his medical degree at Harvard Medical School, Dr. Lee completed a residency in internal medicine at the University of California, San Francisco (UCSF). He subsequently completed a nephrology fellowship at UCSF while simultaneously obtaining a Master of Advanced Study in clinical research from the UCSF Department of Epidemiology and Biostatistics. Dr. Lee is a Fellow of the American Society of Nephrology, a Certified Hypertension Specialist through the American Hypertension Specialist Certification Program, and a member of the American Society of Transplantation. He maintains his clinical practice with the Houston Kidney Consultants.


Assuntos
Cianose/etiologia , Cardiopatias Congênitas/complicações , Nefropatias/etiologia , Rim/fisiopatologia , Cianose/diagnóstico , Cianose/fisiopatologia , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/tratamento farmacológico , Cardiopatias Congênitas/fisiopatologia , Humanos , Rim/efeitos dos fármacos , Rim/patologia , Nefropatias/diagnóstico , Nefropatias/tratamento farmacológico , Nefropatias/fisiopatologia , Prognóstico , Fatores de Risco
12.
Kyobu Geka ; 72(8): 581-585, 2019 Aug.
Artigo em Japonês | MEDLINE | ID: mdl-31353348

RESUMO

Right pulmonary arteriovenous fistula(PAVF) developed in a 18-month-old girl after total cavopulmonary shunt(TCPS) operation. The patient was diagnosed as polysplenia, single atrium, single ventricle, dextrocardia, pulmonary atresia, congenitally complete atrioventricular block, and absent infrahepatic segment of the inferior vena cava with azygous continuation to the right superior vena cava. The hepatic veins were connected to the left-side atrium. At age of 7 month, TCPS operation was performed. Arterial oxygen saturation decreased to 70% at 8 months after TCPS operation. We performed total right heart bypass operation with connected hepatic vein to the azygous vein using a 8-mm ePTFE tube graft, combined with dilatable right pulmonary artery banding( PAB). With PAB on the right pulmonary artery adjacent to the Glenn anastomosis, arterial saturation was 80% and the patient was successfully weaned off cardiopulmonary bypass. At 3 months after TRHB operation, systemic saturation increased to 90%.The right pulmonary artery banding site was successfully dilated by balloon angioplasty.


Assuntos
Anastomose Cirúrgica , Fístula Arteriovenosa/complicações , Derivação Cardíaca Direita , Cardiopatias Congênitas , Artéria Pulmonar/anormalidades , Veias Pulmonares/anormalidades , Fístula Arteriovenosa/cirurgia , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Veias Hepáticas , Humanos , Lactente , Artéria Pulmonar/cirurgia , Veias Pulmonares/cirurgia , Veia Cava Inferior
14.
J Cardiothorac Surg ; 14(1): 104, 2019 Jun 11.
Artigo em Inglês | MEDLINE | ID: mdl-31186037

RESUMO

BACKGROUND: The purpose of this study was to describe our experience with patients who underwent transcatheter closure of a post-operative ventricular septal defect (VSD). METHODS: All patients who underwent transcatheter closure of a VSD after total correction of congenital heart disease since 2012 were enrolled. Medical records were retrospectively reviewed to determine the patients' initial diagnosis, closure device used, and final outcome after device closure. RESULTS: Six patients with a median age of 17.7 years (range: 7 months-48 years) underwent transcatheter closure of an unresolved VSD. The median time interval from the initial corrective surgery to the percutaneous closure procedure was 10.4 years (range: 0.3-33.0 years). The initial diagnoses included tetralogy of Fallot (one patient), VSD (two patients), double outlet of the right ventricle (two patients), and aortic valve stenosis (one patient). The reasons for unresolved VSD (other than leakage) after corrective surgery included previous fenestration (in two patients), and iatrogenic Gerbode shunt (in one patient). Various devices were used, including the Amplatzer duct occluder I, Amplatzer duct occluder II, Amplatzer vascular plug II, and Cocoon membranous VSD occluder. Only one device was used in each patient. There were no major complications associated with the closure procedures. The immediate results were satisfactory. The median follow-up duration was 2.75 years. All cases were successful, with the exception of minimal leak in one patient. CONCLUSIONS: Transcatheter device closure of post-operative VSD can be performed using various device types of devices and is safe and effective. But more experiences are mandatory.


Assuntos
Cateterismo Cardíaco/métodos , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Cardiopatias Congênitas/cirurgia , Comunicação Interventricular/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/complicações , Comunicação Interventricular/etiologia , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/cirurgia , Estudos Retrospectivos , Dispositivo para Oclusão Septal , Resultado do Tratamento , Adulto Jovem
15.
Khirurgiia (Mosk) ; (5): 64-67, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31169821

RESUMO

Hypertrophic cardiomyopathy (HCM) is congenital heart disease with prevalence 1 per 500. There are still unclear issues regarding optimal surgical strategy despite more than 50-year research of this disease. It is presented case report of surgical treatment of patient with obstructive HCM by using of non-standard volume and method of intervention.


Assuntos
Cardiomiopatia Hipertrófica/complicações , Valva Mitral/cirurgia , Obstrução do Fluxo Ventricular Externo/cirurgia , Aorta/cirurgia , Procedimentos Cirúrgicos Cardíacos , Cardiomiopatia Hipertrófica/cirurgia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Humanos , Obstrução do Fluxo Ventricular Externo/etiologia
16.
J Ayub Med Coll Abbottabad ; 31(2): 276-278, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31094131

RESUMO

Accessory mitral valve tissue is a rare congenital anomaly that is commonly incidentally diagnosed. When symptomatic, it tends to present with features of left ventricular outflow (LVOT) obstruction in about two thirds of cases. It is also commonly associated with other congenital anomalies, notably ventricular septal defects. Transthoracic echocardiography is a very useful diagnostic test to make a diagnosis and its widespread use will increase detection of this condition. We are presenting a case of a 29-year-old lady who presented with breathlessness in the third trimester of pregnancy and was subsequently found to have evidence of accessory mitral valve tissue on echocardiography.


Assuntos
Cardiopatias Congênitas , Valva Mitral , Complicações Cardiovasculares na Gravidez , Adulto , Dispneia/etiologia , Ecocardiografia , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Humanos , Valva Mitral/anormalidades , Valva Mitral/diagnóstico por imagem , Gravidez , Complicações Cardiovasculares na Gravidez/diagnóstico por imagem , Complicações Cardiovasculares na Gravidez/fisiopatologia
17.
World J Pediatr Congenit Heart Surg ; 10(3): 292-295, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-31084317

RESUMO

BACKGROUND: Heart failure (HF) is the leading cause of hospitalizations and death in patients with adult congenital heart disease (ACHD). Sacubitril/valsartan is a new agent in the treatment of HF, but its effects have not been assessed in ACHD. METHODS: We retrospectively studied all 15 patients with ACHD at our center who were prescribed sacubitril/valsartan between June 2017 and June 2018. We assessed baseline characteristics and clinical and laboratory changes after initiation of sacubitril/valsartan. Adverse events, including renal function, medication intolerance, and worsening HF were documented. RESULTS: The median age was 53.2 (27.6-83.6) years, with a median follow-up duration of 69 (8-419) days. At baseline, all patients had refractory HF despite guideline-directed medical therapy, with ten (67%) patients as New York Heart Association (NYHA) class II, and five (33%) patients NYHA class III. The medication was discontinued in one (7%) patient secondary to worsening kidney function. No patients reported clinical deterioration; four NYHA class III patients with complex CHD, pulmonary hypertension, and cyanosis reported significant improvement to NYHA class II. Baseline creatinine was 1.1 (0.9-1.7) and two weeks after starting sacubitril/valsartan it was 1.3 (0.8-2.5, P = .22). CONCLUSIONS: Sacubitril/valsartan seems to be well tolerated in patients with ACHD who present with refractory HF symptoms. Patients with complex CHD associated with cyanosis and pulmonary hypertension could benefit the most, but larger studies are needed to assess the safety as well as the effectiveness of sacubitril/valsartan in this patient population.


Assuntos
Aminobutiratos/uso terapêutico , Cardiopatias Congênitas/tratamento farmacológico , Insuficiência Cardíaca/tratamento farmacológico , Volume Sistólico/fisiologia , Tetrazóis/uso terapêutico , Valsartana/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Bloqueadores do Receptor Tipo 1 de Angiotensina II/uso terapêutico , Antagonistas de Receptores de Angiotensina/uso terapêutico , Feminino , Seguimentos , Cardiopatias Congênitas/complicações , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Neprilisina , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
18.
Ter Arkh ; 91(1): 24-31, 2019 Mar 10.
Artigo em Inglês | MEDLINE | ID: mdl-31090367

RESUMO

AIM: Assess the prevalence, clinical course, current therapy, and mortality in patients with pulmonary arterial hypertension (PAH) in the National Registry. MATERIALS AND METHODS: In the prospective study we included patients over 18 years of age with diagnosed PAH [idiopathic PAH (IPAH); Drug- and Toxin-Induced Pulmonary Hypertension; inherited PAH; PAH associated with congenital heart disease (PAH-CHD); PAH associated with systemic connective tissue disease (PAH-CTD); PAH associated with HIV infection (PAH-HIV); with portal pulmonary hypertension (portoPAH)]. The observation was carried out in 15 expert centers of Russia from 01.01.2012 to 31.12.2017. RESULTS: Our registry included 470 patients with PAH: IPAH - 41.5%, PAH-CHD - 36%, PAH-CTD - 19.5%, inherited PAH - 0.4%, portoPAH - 1.9%, PAH-HIV - 0.4%, Drug- and Toxin-Induced PAH - 0.4%. The prevalence among women was 84%. The mean age at the time of patient enrollment in the registry for the overall group of PAH was 42.7±15.3 years. The distance in the 6-minute walking test was 361.3±129.3 m. Among all patients with PAH, 65% had functional class (FC) III/IV at the time of diagnosis, among IPAH - 62%. 69.9% received PAH-specific therapy, of which 62.1% - monotherapy, 32.7% - dual combination therapy, and 5.2% triple therapy. Sildenafil is the most commonly prescribed drug in the regimen of monotherapy. 31.6% of patients were treated with bosentan, 6.4% - riociguat, 3.4% - ambrisentan, 2.1% - macitentan and 2.0% iloprost. Survival of patients with PAH was 98.9% at 1 year of follow-up, 94.1% at 3 years and 86.0% at 5 years. CONCLUSION: The registry data indirectly indicates the need to increase efforts aimed at improving the diagnosis of systemic connective tissue diseases in adults, as well as congenital heart defects in children for timely surgical treatment. In recent years, PAH-specific drugs of the new generation have been introduced into clinical practice, but currently in Russia there are no parenteral prostanoids, which are recommended for the most severe patients.


Assuntos
Anti-Hipertensivos/uso terapêutico , Hipertensão Pulmonar/tratamento farmacológico , Artéria Pulmonar/fisiopatologia , Adulto , Idoso , Feminino , Cardiopatias Congênitas/complicações , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/mortalidade , Pessoa de Meia-Idade , Estudos Prospectivos , Sistema de Registros , Federação Russa/epidemiologia , Taxa de Sobrevida
20.
J Pediatr Surg ; 54(5): 920-924, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30954228

RESUMO

PURPOSE: The purpose of this study was to determine the accuracy of fetal echocardiogram (ECHO) for detecting cardiac structural anomalies that may impact Extracorporeal Membrane Oxygenation (ECMO) candidacy in infants with Congenital Diaphragmatic Hernia (CDH). METHODS: A retrospective review was performed on fetuses with CDH (January 2007-June 2017). Inclusion criteria were inborn and at least one prenatal and postnatal ECHO. ECHOs were evaluated for structural heart defects. Primary outcomes were accuracy of prenatal fetal ECHO and identify differences between prenatal and postnatal ECHO. Descriptive statistics and Chi-square analysis were performed. RESULTS: 131 inborn patients were identified. Mean gestational age of fetal ECHO was 26.6 ±â€¯5.5 weeks. The median time to postnatal ECHO was DOL 1 [0-30]. Fetal ECHO had 92% accuracy, 83% sensitivity, 93% specificity, PPV of 95%, NPV of 92%, and a 90% accuracy for visualization of at least one pulmonary vein into the left atrium on the contralateral (non-CDH) side. Thirty-five percent of patients received ECMO, and 26% had an associated cardiac anomaly. All ECMO patients had an accurate structural fetal ECHO. CONCLUSION: Fetal ECHO is sufficient for identifying major structural heart defects at large volume centers with trained pediatric cardiologists and may be used to guide clinical management, particularly in regards to ECMO candidacy. LEVEL OF EVIDENCE: Level III.


Assuntos
Ecocardiografia , Oxigenação por Membrana Extracorpórea , Cardiopatias Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/terapia , Ultrassonografia Pré-Natal , Feminino , Idade Gestacional , Cardiopatias Congênitas/complicações , Hérnias Diafragmáticas Congênitas/complicações , Humanos , Recém-Nascido , Masculino , Seleção de Pacientes , Período Pós-Parto , Valor Preditivo dos Testes , Estudos Retrospectivos
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