Dupla via de saída de ventrículo único do tipo esquerdo: uma cardiopatia congênita rara

A cardiopatia com dupla via de saída do ventrículo esquerdo (DVSVE) é uma anomalia rara, podendo estar associada com ventrículo esquerdo único, o que a torna mais incomum. Lactente, 6 meses, sexo feminino, admitida em pronto socorro com história de dificuldade de ganho de peso, cansaço às mamadas e atraso no desenvolvimento neuropsicomotor. À ausculta cardíaca apresentava bulhas hiperfonéticas e sopro holossistólico panfocal, mais audível em borda esternal esquerda. Ictus cordis palpável e propulsivo. Apresentava aumento do diâmetro anteroposterior torácico, com retração intercostal e subcostal. Radiografia de tórax demonstrou aumento da área cardíaca e da trama vascular; eletrocardiograma evidenciou sobrecarga atrial e ventricular esquerdas. O ecocardiograma demonstrou situs solitus em levocardia com ventrículo único tipo esquerdo, câmara ventricular direita rudimentar, com dupla via de saída de ventrículo esquerdo, aorta anterior e artéria pulmonar posterior dilatada. Mantida em internação para aguardar correção cirúrgica, evoluiu com quadro de pneumonia nosocomial, descompensação cardiovascular e respiratória, com necessidade de intubação orotraqueal e ventilação mecânica, tendo como desfecho o óbito. A DVSVE é uma entidade heterogênea com diversas variações anatômicas, sendo um delas o ventrículo único. A coexistência dessas associações se torna rara e pouco descrita na literatura. A avaliação do recém-nascido com suspeita de cardiopatia deve ser realizada de maneira cautelosa. O ecocardiograma permite a confirmação diagnóstica. Crianças com cardiopatia congênita apresentam maior risco de complicações infecciosas, sobretudo naquelas com hiperfluxo pulmonar. O tratamento é paliativo e consiste em intervenção cirúrgica. O diagnóstico precoce de cardiopatias congênitas passíveis de intervenção é fundamental para que seja programada a correção cirúrgica o mais breve possível, a fim de promover maior sobrevida ao paciente e reduzir a morbimortalidade. Palavras-chave: Cardiopatias; Anomalia Congênita; Ventrículos do Coração. ABSTRACT Double outlet left ventricle heart disease is a rare anomaly and may be associated with a single ventricle of the left type, which makes it more uncommon. Infant, 6 months, female, admitted in the emergency room with history of difficulty gaining weight, tiredness to feedings and delayed neuropsychomotor development. Cardiac auscultation presented hyperphonectic sounds and panfocal holosystolic murmur, more audible at the left sternal border. Palpable and propulsive Ictus cordis. Thoracic diameter increased with intercostal and subcostal retraction. Chest X-ray showing increased cardiac area and vascular weave; Electrocardiogram showed atrial and left ventricular overload. The echocardiogram showed situs solitus in levocardia with single left ventricle, rudimentary right ventricle chamber, with left ventricular outflow tract, anterior aorta and dilated posterior pulmonary artery. Maintained in hospital to await surgical correction, evolution with hospitable pneumonia, cardiovascular and respiratory decompensating, requiring orotracheal intubation and mechanical ventilation, with the outcome of death. The LVEDV is a heterogeneous entity with several anatomical variations, one of them being the single ventricle. A coexistence associates becomes rare and little described in the literature. An evaluation of the newborn with suspected cardiopathy should be performed with caution. The echocardiogram allows diagnostic confirmation. Children with congenital heart disease have an increased risk of infectious complications, especially in those with pulmonary hyperflow. The treatment is palliative and consists of surgical intervention. The prior diagnosis of congenital heart diseases that are amenable to intervention is essential for the timing of surgical correction or as soon as possible, in order to promote greater patient survival and reduce morbidity and mortality. Keywords: Heart Diseases; Congenital Abnormalities; Heart Ventricles.

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The entirely subcutaneous ICDTM system in patients with congenital heart disease: experience from a large single-centre analysis

EP Europace ◽

10.1093/europace/euz190 ◽

2019 ◽

Vol 21 (10) ◽

pp. 1537-1542 ◽

Cited By ~ 1

Author(s):

Kevin Willy ◽

Florian Reinke ◽

Nils Bögeholz ◽

Julia Köbe ◽

Lars Eckardt ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Heart Diseases ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Single Centre ◽

Icd Implantation ◽

Shock Delivery ◽

Ventricular Ejection Fraction

Abstract Aims The subcutaneous implantable cardioverter-defibrillator (S-ICDTM) is an important advance in device therapy for the prevention of sudden cardiac death (SCD). Although current guidelines recommend S-ICDTM use, long-term data are still limited, especially in subgroups such as adult patients with congenital heart diseases. This cohort is of high interest because of the difficult anatomic conditions in these patients. Methods and results All S-ICDTM patients with an underlying congenital heart disease (CHD) resulting in an indication for ICD implantation (n = 20 patients) in our large-scaled single-centre S-ICDTM registry (n = 249 patients) were included in this study. Baseline characteristics, appropriate and inappropriate shocks, and complications were documented in a mean follow-up of 36 months. Primary prevention of SCD was the indication for implantation of an S-ICDTM in six patients (30%). Of all 20 patients with an overall mean age of 40.5 ± 11.5 years, 12 were male (60%). The mean left ventricular ejection fraction was 46.5 ± 11.3%. Nine episodes of ventricular tachycardia (two monomorphic and seven polymorphic) were adequately terminated in three patients (15%). In two patients, T-Wave-Oversensing resulting in an inappropriate shock was observed, which could be managed by changing the sensing vector or activation of the SMART PASSTM filter. There were no S-ICDTM system-related infections. In one patient, surgical revision was necessary due to a persistent haematoma. Conclusion The S-ICDTM seems to be a valuable option for the prevention of SCD in patients with various CHDs and complex anatomical anomalies. The S-ICDTM is safe and works effectively, also in these complex patients. Inadequate shock delivery was rare and could be managed by reprogramming.

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Superior Vena Cava Banding to Facilitate Unilateral Bidirectional Glenn Operation in Patients With Single Ventricle Heart Disease and Bilateral Superior Caval Veins

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135116679824 ◽

2016 ◽

Vol 8 (2) ◽

pp. 215-219 ◽

Cited By ~ 1

Author(s):

Matthew C. Schwartz ◽

David Nykanen ◽

William DeCampli ◽

Kamal Pourmoghadam

Keyword(s):

Heart Disease ◽

Congenital Heart ◽

Single Ventricle ◽

Superior Vena ◽

Vena Cava ◽

Novel Technique ◽

Bidirectional Glenn ◽

Increased Risk ◽

Bridging Vein ◽

Cavopulmonary Connection

Staged palliation to achieve a total cavopulmonary connection is a common treatment strategy in patients with single ventricle congenital heart disease. Patients with bilateral superior caval veins (bilateral SVC) often require the creation of bilateral superior cavopulmonary connections as part of the staged palliation, and these patients are at increased risk of morbidity. We describe a novel technique used in two patients with bilateral SVC and very small (1-2 mm) bridging vein that encouraged bridging vein growth and facilitated creation of a unilateral superior cavopulmonary connection.

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REHABILITATION OF STROKE IN CYANOTIC CONGENITAL SINGLE VENTRICLE HEART DEFECT PATIENT

Journal of medical pharmaceutical and allied sciences ◽

10.22270/jmpas.v10i6.1350 ◽

2021 ◽

Vol 10 (6) ◽

pp. 3762-3765

Author(s):

Ragini Dadgal

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Ischemic Stroke ◽

Congenital Heart ◽

Single Ventricle ◽

Exercise Intolerance ◽

Arterial Ischemic Stroke ◽

Breathing Exercises ◽

Increased Risk ◽

Bed Mobility

Congenital heart disease consists of various conditions including tetralogy of Fallot, ventricular septal defect, Epstein’s anomaly, single ventricle, etc. Among these single ventricles is one of the gravest forms of cyanotic congenital heart disease. The cardiac diagnosis is associated with an increased risk of stroke among children. Pediatric arterial ischemic stroke (AIS) is an important cause of neurologic disease in children causing disability. The 14-year-old patient came to the hospital was presented with left side hemiplegia with severe exercise intolerance due to congenital heart disease. The patient has been advised to undergo Fontan procedure for single-ventricle condition 3 years back, but due to poor socioeconomic status, parents of patients refused to do so. The primary goal was to improve bed mobility and trunk balance without developing symptoms of exercise intolerance. The intervention was started with deep and segmental breathing exercises. Proprioceptive neuromuscular facilitation and constrained induced movement therapy were added in the program in addition to passive and active movements, bed mobility, functional reeducation, trunk control exercises, and balance exercises. Combinations of all of the above therapeutic approaches lead to increased functional independence in the patient. This case reports the effectiveness of a rehabilitation program for pediatric arterial ischemic stroke with preventive guidelines for exercise intolerance.

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Congenital Heart Disease in non-Diabetic Large-for-Gestational-Age (LGA) Neonates

Cardiovascular & Haematological Disorders - Drug Targets ◽

10.2174/1871529x20666201216170012 ◽

2020 ◽

Vol 20 ◽

Author(s):

Majid Firouzi ◽

Hamidreza Sherkatolabbasieh ◽

Alireza Nezami ◽

Shiva Shafizadeh

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Birth Weight ◽

Gestational Age ◽

Congenital Heart ◽

Heart Diseases ◽

Large For Gestational Age ◽

Congenital Heart Diseases ◽

Increased Risk ◽

Heart Abnormalities

Background: Congenital heart diseases are the most prevalent congenital abnormalities in the neonates, caused by the environmental and genetic factors and contribute to the leading cause of death. The aim of this study is to evaluate the relationship between neonates with large for gestational age and increased risk of congenital heart diseases among nondiabetic mothers. Methods: In this study, 179 neonates with large for gestational age in Khorramabad were enrolled where heart abnormalities were evaluated using echocardiography. Results: 87 neonates had more than 4000 g of the birth weight with no heart abnormalities and 92 (51%) macrosomic neonates had congenital heart diseases. Statistical analysis revealed that there was a significant relationship between birth weight and increased risk of acquiring congenital heart disease between the two groups. There was no significant relationship between birth weight, maternal age, gender, labor type and blood group between the two groups. The highest incidence of congenital heart anomalies was related to 38% of arterial septal defect (ASD) and 15.2% of ASD and VSD, respectively Conclusion: The most prevalent abnormality was arterial septal ASD. None of these abnormalities were associated with maternal age, birth weight and neonate gender. Future studies for congenital heart disease and neonatal birth weight are therefore, recommended.

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P5353Pregnancy in congenital heart disease - risk stratificaton

European Heart Journal ◽

10.1093/eurheartj/ehz746.0320 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

S Pataki ◽

M Szegedi ◽

A Temesvari ◽

L Ablonczy ◽

P Andreka ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

High Risk ◽

Premature Birth ◽

Congenital Heart ◽

Left Ventricular ◽

High Risk Pregnancy ◽

Cardiac Events ◽

Risk Pregnancy ◽

Increased Risk

Abstract Introduction Maternal congenital heart disease is a leading cause of peripartum maternal mortality, which is also associated with increased risk of fetal morbidity and mortality. We aimed to provide risk stratification for congenital heart disease patients using the recently introduced CARPREG (Cardiac Disease in Pregnancy) II score at our Institute. Patients and methods We enrolled 191 pregnant women with congenital heart disease (mean age at pregnancy 29.2±5,7 years). We recorded all foetal (premature birth, abortion, congenital heart abnormalities) and maternal (postpartum heart failure, stroke, hypertension) events in 276 pregnancies. High-risk pregnancy was defined as a CARPREG II score of 4≤. Results High-risk pregnancy was detected in 14.1%. We found fetal and maternal events in 13.8% and 14.5% of all pregnancies, respectively, which both were more prevalent in the high-risk group (p<0.001). No maternal death occurred, whereas premature birth and abortion was detected in 7.6 and 2.9%, respectively, both complications were more frequent in the high-risk patient population as compared to the lower risk patients (30.8 vs. 7.2%, p<0.001). In univariate analysis, CARPREG II of 4≤ was significantly associated with fetal (p<0.001, OR: 4.2) and maternal (p<0.001, OR: 5.3) events. Risk factors of the CARPREG II were further analyzed using multivariate logistic regression analysis: prior cardiac events or arrhythmias (p=0.027) and cyanosis (p=0.026) were independent predictors of fetal complications. Left ventricular outflow tract obstruction (p<0.001), cardiac interventions (p=0.024) and prior cardiac events or arrhythmias (p=0.001) demonstrated significant association with maternal events. Conclusion The CARPREG II could help in the identification of high-risk pregnancies and thus aids the selection of patients for strict monitoring and special therapies. In line with previous studies we detected higher rates of fetal and maternal events among patients with CARPREG II of 4≤ score.

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Attention Deficit Hyperactivity Disorder (ADHD) in Patients With Congenital Heart Disease (CHD)

10.21203/rs.3.rs-663884/v1 ◽

2021 ◽

Author(s):

Hassan Mottaghi Moghaddam Shahri ◽

Mahboubeh Eslamzadeh ◽

Aazam Sadat Heydari Yazdi

Keyword(s):

Attention Deficit Hyperactivity Disorder ◽

Congenital Heart Disease ◽

Heart Disease ◽

Attention Deficit ◽

Congenital Heart ◽

Heart Diseases ◽

Developmental Problems ◽

Hyperactivity Disorder ◽

Increased Risk ◽

The Mean

Abstract BackgroundIt is established that children with congenital heart disease (CHD) are more susceptible for suffering deficiencies in intellectual functioning, developmental problems, and academic performance difficulties. Neurodevelopmental disabilities, particularly executive function impairments, are currently the most prevalent long-term morbidity in the population with CHD. The aim of this study was to investigate the frequency of Attention deficit hyperactivity disorder (ADHD) in children with CHD. MethodsThis was a retrospective cohort study, which was performed between 2002-2018 in patients with CHD referred to Imam Reza hospital, Mashhad, Iran. Using the census method, all files for which ADHD diagnosis has been made according to DSM-IV or DSM-V criteria should be included in the study. Diagnosis of CHD was performed clinically and using an echocardiographic machine by an experienced pediatric cardiologist. Heart diseases were divided into two important (major) and non-important (minor) categories based on the need for follow-up and intervention. Demographic, clinical and para-clinical data of patients as well as the type of heart intervention were collected and analyzed.Results136 patients were enrolled in the study. The mean age of participants in the study was 59.12± 45.84 months at the time of diagnosis. Abnormal electroencephalogram (EEG) was reported in three cases (2.2%). There was significant correlation between prematurity and developmental delay (P=0.01). The mean of age was significantly different in patients with minor rather than patients with major cardiac disorders (P<0.05). The prevalence of ADHD in CHD population was high (31.6%).ConclusionThis study demonstrated that children born with CHD are at increased risk of suffering from ADHD.

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Down’s Syndrome with Congenital Heart Disease: Our Surgical Experience

Cardiovascular Journal ◽

10.3329/cardio.v13i1.50562 ◽

2020 ◽

Vol 13 (1) ◽

pp. 35-39

Author(s):

Md Zahidul Islam ◽

Sakila Israt Jahan ◽

Shahriar Moinuddin ◽

Khondokar Shamim Shahriar Ziban Rushel ◽

Shafiqul Islam ◽

...

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Cardiac Surgery ◽

Heart Disease ◽

Congenital Heart ◽

Heart Diseases ◽

Down's Syndrome ◽

Down’S Syndrome ◽

Surgical Correction ◽

Pace Maker

Background: Our objective was to analyze the outcome of patients of Down’s syndrome with congenital heart diseases undergoing cardiac surgery. Methods: This was a retrospective study conducted between January 2013 and June 2019. 49 consecutive patients with Down’s syndrome with congenital heart disease admitted in pediatric cardiac surgery unit at National Institute of Cardiovascular Diseases (NICVD). Patients were followed up postoperatively for in-hospital outcome. Results: Among 49 patients the heart lesion ranked in incidence as follows- VSD 24(48.97%), AV canal defect 12(24.48%), TOF 6(12.24%), PDA 6(12.24%) and ASD 1(2.04%). Pulmonary hypertension was found in 63.25% patients. Moderate pulmonary hypertension was most common, found in 18(38.66%) patients. Severe and mild pulmonary hypertension was found in 10(32.38%) and 3(9.67%) patients respectively. All the patients had undergone surgical correction. The postoperative period was complicated in 44.89% of patients. The most frequent complication was pulmonary infection 20.40%, Wound infection 6.12% and low output syndrome 6.12% were the next. One patient had postoperative heart block, needed permanent pace maker implantation. In-hospital mortality was 12.24%. Conclusion: Patients with Down’s syndrome with congenital heart disease undergoing surgical correction had an acceptable postoperative morbidities and mortality. Cardiovasc. j. 2020; 13(1): 35-39

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Heart lesions in individuals with arterial hypertension of various risk levels: the possibility of diagnosis and treatment

Cardiosomatics ◽

10.26442/22217185.2020.2.200228 ◽

2020 ◽

Vol 11 (2) ◽

pp. 35-38

Author(s):

Anatolii N. Britov ◽

Elena M. Platonova ◽

Nina A. Eliseeva

Keyword(s):

Heart Disease ◽

Ventricular Hypertrophy ◽

Heart Diseases ◽

Hypertensive Heart Disease ◽

Left Ventricular ◽

Diagnostic Methods ◽

Risk Levels ◽

Increased Risk ◽

Outpatient Practice ◽

Heart Lesions

Based on scientific literature and own data, the article discusses the problem of hypertensive heart disease in real outpatient practice and especially in conducting preventive examinations in the working population. It is shown that the use of modern diagnostic methods, in particular electrocardiography with dopplerography, including pulse-wave tissue mode, should be an integral study included in the professional examination, as well as medical examination, which covers an increasing number of the population of our country. Echocardiography should be performed to clarify the cardiovascular risk and confirm the electrocardiographic diagnosis of left ventricular hypertrophy (LVH), left atrial dilatation, or suspected heart diseases (coronary heart disease, atrial fibrillation, other rhythm disorders). It has been shown that concentric LVH is the strongest predictor of an increased risk of heart failure, although the degree of LVH is not always directly related to the degree of hypertension and the duration of the disease.

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A pilot study of expenditures on, and utilization of resources in, health care in adults with congenital heart disease

Cardiology in the Young ◽

10.1017/s1047951101000336 ◽

2001 ◽

Vol 11 (3) ◽

pp. 301-313 ◽

Cited By ~ 35

Author(s):

Philip Moons ◽

Kaat Siebens ◽

Sabina De Geest ◽

Ivo Abraham ◽

Werner Budts ◽

...

Keyword(s):

Health Care ◽

Congenital Heart Disease ◽

Heart Disease ◽

Health Insurance ◽

Pilot Study ◽

General Population ◽

Congenital Heart ◽

Heart Diseases ◽

Left Ventricular ◽

Congenital Heart Diseases

Background: Congenital cardiac disease may be a chronic condition, necessitating life-long followup for a substantial proportion of the patients. Such patients, therefore, are often presumed to be high users of resources for health care. Information on utilization of resources in adults with congenital heart disease, however, is scarce. Methods: This retrospective pilot study, performed in Belgium, investigated 192 adults with congenital heart disease to measure the annual expenditures and utilization of health care and compared the findings with data from the general population. We also sought to explore demographic and clinical parameters as predictors for the expenditures. Results: Hospitalization was documented in 20.3% of the patients, with a median length of stay of 5 days. The overall payment by health insurance associations in 1997 was 1794.5 ECU per patient, while patients paid on average 189.5 ECU out-of-pocket. For medication, the average reimbursement and out-of-pocket expenses were estimated at 78 ECU and 20 ECU, respectively. Expenditures for patients with congenital heart disease were considerably higher than the age and gender-corrected expenditures for the general population (411.7 ECU), though this difference was accounted for by only one-eighth of the cohort of those with congenital heart disease. In general, higher expenditures were associated with abnormal left ventricular end-diastolic diameter, female gender, functional impairment and higher age, although the explained variance was limited. Conclusion: Our study has provided pilot data on the economic outcomes for patients with congenital heart diseases. We have identified parameters that could predict expenditure, but which will have to be examined in future research. This is needed to develop guidelines for health insurance for those with congenital heart diseases.

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Ablation of Atrial Fibrillation in Patients with Congenital Heart Disease

Arrhythmia & Electrophysiology Review ◽

10.15420/2017.2017.15.1 ◽

2017 ◽

Vol 6 (4) ◽

pp. 191 ◽

Cited By ~ 7

Author(s):

Marwan M Refaat ◽

Jad Ballout ◽

Moussa Mansour ◽

◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Catheter Ablation ◽

Congenital Heart ◽

Heart Diseases ◽

Superior Vena ◽

Vena Cava ◽

Surgical Techniques ◽

Ebstein Anomaly ◽

Increased Risk

With improved surgical techniques and medical management for patients with congenital heart diseases, more patients are living longer and well into adulthood. This improved survival comes with a price of increased morbidity, mainly secondary to increased risk of tachyarrhythmias. One of the major arrhythmias commonly encountered in this subset of cardiac patients is AF. Similar to the general population, the risk of AF increases with advancing age, and is mainly secondary to the abnormal anatomy, abnormal pressure and volume parameters in the hearts of these patients and to the increased scarring and inflammation seen in the left atrium following multiple surgical procedures. Catheter ablation for AF has been shown to be a very effective treatment modality in patients with refractory AF. However, data and guidelines regarding catheter ablation in patients with congenital heart disease are not well established. This review will shed light on the procedural techniques, success rates and complications of AF catheter ablation in patients with different types of CHD, including atrial septal defects, tetralogy of Fallot, persistent left superior vena cava, heterotaxy syndrome and atrial isomerism, and Ebstein anomaly.

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