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Congenital, Hereditary, and Neonatal Diseases and Abnormalities - Top 30 Publications

Two Phase 3 Trials of Dupilumab versus Placebo in Atopic Dermatitis.

Two Phase 3 Trials of Dupilumab versus Placebo in Atopic Dermatitis.

Adherence and Recursive Perception Among Young Adults with Cystic Fibrosis.

Adherence to prescribed treatment is a pressing issue for adolescents and young adults with cystic fibrosis (CF). This paper presents two narratives from the thematic analysis of unstructured interviews with 14 adolescents, young adults, and older adults living with CF. Through a new identity-based framework termed recursive perception that draws focus on how an individual perceives how others view them, it explores the social context of adherence and self-care among young adults with CF. It demonstrates that an individual's understanding of self and desire to maintain a certain image for peers can be deeply embedded in adherence and self-care patterns, leading individuals to feel they need to choose between tending to their health needs and living their lives. This suggests that current biomedical innovation in CF care must be complemented with renewed efforts to find effective means to empower young adults with CF to successfully navigate the social challenges of their illness and avoid the pitfalls of nonadherence that can lead to a permanent worsening of their health condition.

Anterior condylar confluence dural arteriovenous fistula: a rare cause of hoarseness.

Hoarseness secondary to an anterior condylar confluence (ACC) dural arteriovenous fistula (DAVF) has not been previously described. We present a 58-year-old patient with a 3-week history of progressive unilateral left-sided headaches and hoarseness. Nasolaryngoscopy and CT neck showed the presence of unilateral vocal cord palsy with no identifiable cause along the expected course of the recurrent laryngeal nerve. MRI revealed an incidental finding of abnormal serpiginous vessels in the left hypoglossal canal which led to a diagnostic cerebral angiogram, confirming the presence of an ACC DAVF. The patient underwent transvenous coil embolisation with subsequent resolution of arteriovenous shunting and symptoms. Follow-up MRI at 6 months showed no recurrence and there was complete resolution of clinical symptoms.

Spinal arachnoid cysts - our experience and review of literature.

Arachnoid cysts are discrete pockets of CSF or CSF-like fluid found adjacent to normal CSF spaces, either spinal or cranial. Spinal arachnoid cysts (SAC) are most commonly extradural, however intradural or perineural are also described.

Views and Decisions of Physicians in Encountering Neonates with Poor Prognosis.

With the development of neonatal intensive care units (NICUs), new issues have emerged for physicians working in this area, including the ethical aspects of providing invasive and advanced care to neonates with extremely poor prognosis. This research was undertaken with the aim of investing the factors affecting physicians' practice in management of newborns in such complicated circumstances.

Evaluation of Initial Respiratory Support Strategies in VLBW Neonates with RDS.

Non-invasive ventilation (NIV) has brought about a significant change in care and treatment of respiratory distress syndrome (RDS) in very low birth weight (VLBW) neonates. The present study was designed and conducted to evaluate different strategies of initial respiratory support (IRS) in VLBW neonates hospitalized in the neonatal intensive care unit (NICU).

Orthostatic Intolerance and Postural Orthostatic Tachycardia Syndrome in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome, Hypermobility Type: Neurovegetative Dysregulation or Autonomic Failure?

Background. Joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type (JHS/EDS-HT), is a hereditary connective tissue disorder mainly characterized by generalized joint hypermobility, skin texture abnormalities, and visceral and vascular dysfunctions, also comprising symptoms of autonomic dysfunction. This study aims to further evaluate cardiovascular autonomic involvement in JHS/EDS-HT by a battery of functional tests. Methods. The response to cardiovascular reflex tests comprising deep breathing, Valsalva maneuver, 30/15 ratio, handgrip test, and head-up tilt test was studied in 35 JHS/EDS-HT adults. Heart rate and blood pressure variability was also investigated by spectral analysis in comparison to age and sex healthy matched group. Results. Valsalva ratio was normal in all patients, but 37.2% of them were not able to finish the test. At tilt, 48.6% patients showed postural orthostatic tachycardia, 31.4% orthostatic intolerance, 20% normal results. Only one patient had orthostatic hypotension. Spectral analysis showed significant higher baroreflex sensitivity values at rest compared to controls. Conclusions. This study confirms the abnormal cardiovascular autonomic profile in adults with JHS/EDS-HT and found the higher baroreflex sensitivity as a potential disease marker and clue for future research.

Bioinformatics Analysis Reveals MicroRNAs Regulating Biological Pathways in Exercise-Induced Cardiac Physiological Hypertrophy.

Exercise-induced physiological cardiac hypertrophy is generally considered to be a type of adaptive change after exercise training and is beneficial for cardiovascular diseases. This study aims at investigating exercise-regulated microRNAs (miRNAs) and their potential biological pathways. Here, we collected 23 miRNAs from 8 published studies. MirPath v.3 from the DIANA tools website was used to execute the analysis, and TargetScan was used to predict the target genes. Kyoto Encyclopedia of Genes and Genomes (KEGG) and Gene Ontology (GO) analyses were performed to identify potential pathways and functional annotations associated with exercise-induced physiological cardiac hypertrophy. Various miRNA targets and molecular pathways, such as Fatty acid elongation, Arrhythmogenic right ventricular cardiomyopathy (ARVC), and ECM-receptor interaction, were identified. This study could prompt the understanding of the regulatory mechanisms underlying exercise-induced physiological cardiac hypertrophy.

Usage of 3D models of tetralogy of Fallot for medical education: impact on learning congenital heart disease.

Congenital heart disease (CHD) is the most common human birth defect, and clinicians need to understand the anatomy to effectively care for patients with CHD. However, standard two-dimensional (2D) display methods do not adequately carry the critical spatial information to reflect CHD anatomy. Three-dimensional (3D) models may be useful in improving the understanding of CHD, without requiring a mastery of cardiac imaging. The study aimed to evaluate the impact of 3D models on how pediatric residents understand and learn about tetralogy of Fallot following a teaching session.

Use of a biodegradable, oversized stent in a child with tracheomalacia secondary to vascular external compression.

We describe the implantation of an absorbable, custom-made stent of polydioxanone to treat tracheomalacia in a 5-month-old patient with extrinsic compression by a double aortic arch. The use of an absorbable, oversized stent treated the tracheal collapse caused by vascular compression, avoided removal procedures, and allowed the infant's growth. The use of an oversized stent prevented stent migration and gave minimal problems of granulation.

Reversal of severe mitral regurgitation by device closure of a large patent ductus arteriosus in a premature infant.

We report a critically ill premature infant with severe mitral valve regurgitation associated with pulmonary hypertension and a severely dilated left atrium from a large patent ductus arteriosus. The mitral valve regurgitation improved significantly with normalisation of left atrial size 4 weeks after percutaneous closure of the patent ductus arteriosus. This case highlights the potential reversibility of severe mitral valve regurgitation with treatment of an underlying cardiac shunt.

Percutaneous closure of multiple ventricular septal defects: simultaneous use of muscular ventricular septal defect device and Multi-Fenestrated Septal Occluder - "Cribriform" to close residual ventricular septal defects after complex cardiac surgery in a child.

A male child, with d-transposition of great arteries, a large perimembranous ventricular septal defect, multiple additional ventricular septal defects, small muscle-bound right ventricle, and severe pulmonary stenosis with confluent, moderate-sized branch pulmonary arteries, underwent an emergency right modified Blalock-Taussig shunt on day 15 of life and réparation à l'étageventriculaire procedure with ventricular septal defect closure with takedown of the Blalock-Taussig shunt at 2.5 years of age. On follow-up, he showed a moderate residual upper ventricular septal defect and multiple apical ventricular septal defects, mild mid-right pulmonary artery stenosis, free pulmonary regurgitation, and right ventricular dysfunction. Surgical re-intervention was deemed extremely risky, the upper muscular ventricular septal defect was closed using an 8-mm Amplatzer Muscular Ventricular Septal Defect Occluder Device, and an 18 mm Amplatzer Multi-Fenestrated Septal Occluder - Cribriform was used for the multiple apical muscular ventricular septal defects. After 1 year, his right pulmonary artery stenosis worsened, for which right pulmonary artery angioplasty was carried out using an 8×20 mm cutting balloon followed by a 10×20 mm Tyshak II balloon. This is the only case reported for the paediatric age group using a cribriform septal occluder device for percutaneous closure of multiple apical ventricular septal defects.

Early hybrid approach and enteral feeding algorithm could reduce the incidence of necrotising enterocolitis in neonates with ductus-dependent systemic circulation.

The reported incidence of necrotising enterocolitis in neonates with complex CHD with ductus-dependent systemic circulation ranges from 6.8 to 13% despite surgical treatment; the overall mortality is between 25 and 97%. The incidence of gastrointestinal complications after hybrid palliation for neonates with ductus-dependent systemic circulation still has to be defined, but seems comparable with that following the Norwood procedure.

Abnormalities in serum biomarkers correlate with lower cardiac index in the Fontan population.

Fontan survivors have depressed cardiac index that worsens over time. Serum biomarker measurement is minimally invasive, rapid, widely available, and may be useful for serial monitoring. The purpose of this study was to identify biomarkers that correlate with lower cardiac index in Fontan patients. Methods and results This study was a multi-centre case series assessing the correlations between biomarkers and cardiac magnetic resonance-derived cardiac index in Fontan patients ⩾6 years of age with biochemical and haematopoietic biomarkers obtained ±12 months from cardiac magnetic resonance. Medical history and biomarker values were obtained by chart review. Spearman's Rank correlation assessed associations between biomarker z-scores and cardiac index. Biomarkers with significant correlations had receiver operating characteristic curves and area under the curve estimated. In total, 97 cardiac magnetic resonances in 87 patients met inclusion criteria: median age at cardiac magnetic resonance was 15 (6-33) years. Significant correlations were found between cardiac index and total alkaline phosphatase (-0.26, p=0.04), estimated creatinine clearance (0.26, p=0.02), and mean corpuscular volume (-0.32, p<0.01). Area under the curve for the three individual biomarkers was 0.63-0.69. Area under the curve for the three-biomarker panel was 0.75. Comparison of cardiac index above and below the receiver operating characteristic curve-identified cut-off points revealed significant differences for each biomarker (p<0.01) and for the composite panel [median cardiac index for higher-risk group=2.17 L/minute/m2 versus lower-risk group=2.96 L/minute/m2, (p<0.01)].

Recognizing CADASIL: a Secondary Cause of Migraine with Aura.

CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is an important disease to consider in the differential diagnosis of migraine with aura. This review examines current literature regarding migraine in CADASIL, as well as diagnostic and treatment modalities.

Existence of HbF Enhancer Haplotypes at HBS1L-MYB Intergenic Region in Transfusion-Dependent Saudi β-Thalassemia Patients.

Background and Objectives. β-Thalassemia and sickle cell disease are genetic disorders characterized by reduced and abnormal β-globin chain production, respectively. The elevation of fetal hemoglobin (HbF) can ameliorate the severity of these disorders. In sickle cell disease patients, the HbF level elevation is associated with three quantitative trait loci (QTLs), BCL11A, HBG2 promoter, and HBS1L-MYB intergenic region. This study elucidates the existence of the variants in these three QTLs to determine their association with HbF levels of transfusion-dependent Saudi β-thalassemia patients. Materials and Methods. A total of 174 transfusion-dependent β-thalassemia patients and 164 healthy controls from Eastern Province of Saudi Arabia were genotyped for fourteen single nucleotide polymorphisms (SNPs) from the three QTL regions using TaqMan assay on real-time PCR. Results. Genotype analysis revealed that six alleles of HBS1L-MYB QTL (rs9376090C p = 0.0009, rs9399137C p = 0.008, rs4895441G p = 0.004, rs9389269C p = 0.008, rs9402686A p = 0.008, and rs9494142C p = 0.002) were predominantly associated with β-thalassemia. In addition, haplotype analysis revealed that haplotypes of HBS1L-MYB (GCCGCAC p = 0.022) and HBG2 (GTT p = 0.009) were also predominantly associated with β-thalassemia. Furthermore, the HBS1L-MYB region also exhibited association with the high HbF cohort. Conclusion. The stimulation of HbF gene expression may provide alternative therapies for the amelioration of the disease severity of β-thalassemia.

Anomalous origin of the circumflex coronary artery presenting with ventricular fibrillation cardiac arrest.

We report a case of an incidental finding of an anomalous left circumflex coronary artery arising from the right pulmonary artery that effectuated a ventricular fibrillation cardiac arrest in a woman aged 34 years. This rarity was detected during routine work-up to delineate the cause of this arrhythmia. Our patient had a background of double-outlet right ventricle and a ventricular septal defect, which was repaired with a Dacron patch and a left ventricle patch over to the aorta at age 14 months. Angiographic study at the time of her presentation showed anomalous origin of the left circumflex artery originating from the right pulmonary artery; this was discussed in multispecialty team meeting and surgical intervention was recommended; eventually, surgery was performed with reimplantation of the anomalous circumflex artery into the ascending aorta. We highlight the importance of early angiographic studies in patients with known congenital heart defects and emphasise the optimal strategy of treatment.

Myxoedema in a patient with achondroplasia in rural area of Guatemala.

A 43-year-old indigenous Guatemalan woman with achondroplasia presented to our clinic with chronic fatigue and generalised oedema. She had limited contact with the formal healthcare system. However, 1 year prior, she had sought medical evaluation from a private physician. Her symptoms had been attributed to a combination of heart failure and physical disability due to the musculoskeletal complications of her achondroplasia. She was lost to follow-up due to inability to pay for further testing or treatment. On initial laboratory evaluation in our clinic, she was found to have a thyrotropin level greater than assay. With initiation of oral levothyroxine supplementation, her dyspnoea and oedema completely resolved. The case illustrates how indigenous patients in rural Guatemala experience many barriers to accessing high-quality medical care. As a result, presentations of common illnesses are often very advanced and definitive diagnoses and treatments are frequently delayed.

CDC Grand Rounds: Public Health Strategies to Prevent Neonatal Abstinence Syndrome.

Neonatal abstinence syndrome (NAS) is a drug withdrawal syndrome that most commonly occurs in infants after in utero exposure to opioids, although other substances have also been associated with the syndrome (1). NAS usually appears within 48-72 hours of birth with a constellation of clinical signs, including central nervous system irritability (e.g., tremors), gastrointestinal dysfunction (e.g., feeding difficulties), and temperature instability (1) (Box 1). Opioid exposure during pregnancy might result from clinician-approved use of prescription opioids for pain relief; misuse or abuse of prescription opioids; illicit use (e.g., heroin); or medication-assisted treatment (MAT) of opioid use disorder (2) (Box 2).

Macrosomia, shoulder dystocia and elongation of the brachial plexus: what is the role of caesarean section?

The delivery of a macrosomic infant is associated with a higher risk for maternofoetal complications. Shoulder dystocia is the most feared fetal complication, leading sometimes to a disproportionate use of caesarean section. This study aims to evaluate the interest of preventive caesarean section. We conducted a retrospective study of 400 macrosomic births between February 2010 and December 2012. We also identified cases of infants with shoulder dystocia occurred in 2012 as well as their respective birthweight. Macrosomic infants weighed between 4000g and 4500g in 86.25% of cases and between 4500 and 5000 in 12.25% of cases. Vaginal delivery was performed in 68% of cases. Out of 400 macrosomic births, 9 cases with shoulder dystocia were recorded (2.25%). All of these cases occurred during vaginal delivery. The risk for shoulder dystocia invaginal delivery has increased significantly with the increase in birth weight (p <10-4). The risk for elongation of the brachial plexus was 11 per thousand vaginal deliveries of macrosomic infants. This risk was not correlated with birthweight (p = 0.38). The risk for post-traumatic sequelae was 0.71%. Shoulder dystocia affectd macrosoic infants in 58% of cases. Shoulder dystocia is not a complication exclusively associated with macrosomia. Screening for risky deliveries and increasing training of obstetricians on maneuvers in shoulder dystocia seem to be the best way to avoid complications.

Transfer and transport of newborn babies in vital distress in Yaoundé, Cameroon: situational analysis conducted in a reference hospital.

This study aims to describe transfer modalities of newborn babies in vital distress to the ESSOS Hospital Center in Yaoundé, Cameroon.

Complete androgen insensitivity syndrome or testicular feminization: review of literature based on a case report.

Testicular feminization, or the androgen insensitivity syndrome, is a rare disease. Because of various abnormalities of the X chromosome, a male, genetically XY, has some physical characteristics of a woman or a full female phenotype. Indeed the androgen insensitivity syndrome occurs because of a resistance to the actions of the androgen hormones, which in turn switches the development towards the aspect of a woman. We report a case of complete androgen insensitivity syndrome in a 30 years old woman who presented primary amenorrhea. We aim to improve our knowledge of this illness from the data that provides us this study, and a review of the literature.

Hypertrophic angulation deformity of the basal interventricular septum combined with abnormality of the papillary muscle and chordae tendineae.

A Chinese woman was admitted to our hospital because of syncope. Transthoracic echocardiography revealed a hypertrophic basal interventricular septum of 15 mm with a sharp angle protruding into the left ventricular outflow tract. Moreover, an anomalous anterolateral papillary muscle (maximum width of 11 mm) was inserted into the left ventricular outflow tract, with short chordae tendineae connecting both basal interventricular septum and anterior leaflet of the mitral valve. All of these abnormalities resulted in a left ventricular outflow gradient of 136 mmHg. Surgical septal myectomy of the sharp angle combined with partial papillary muscle resection and removal of the abnormal chordae tendineae was selected to relieve the left ventricular outflow obstruction. This was a rare combination of deformity of the angulation of the focal basal interventricular septum and abnormalities of the papillary muscle and chordae tendineae, which led to left ventricular outflow obstruction.

Schizophrenia-like symptoms in a patient with Leigh syndrome.

Leigh syndrome is a mitochondrial disease characterized by subacute necrotizing encephalomyelopathy. Almost all cases of Leigh syndrome develop at infancy or early childhood and die within several years due to rapidly progressive muscle weakness and respiratory failure. Here, we present a rare case of a patient who developed Leigh syndrome associated with thiamine-responsive pyruvate dehydrogenase-complex deficiency at 2 years of age and has survived to adolescence through effective high dose thiamin therapy. At 15 years of age, the patient presented persecutory delusions and auditory hallucinations, suggesting an association between mitochondrial dysfunction and schizophrenia-like psychotic symptoms.

Expansion of the phenotypic spectrum in three families of methyl CpG-binding protein 2 duplication syndrome.

The methyl CpG-binding protein 2 duplication syndrome (OMIM #300260) is characterized by hypotonia, developmental delay, spasticity, seizures, and recurrent infections. It is fully penetrant in males and the females can have varied manifestations because of skewed X-inactivation. The size of the duplication can range from 0.2 Mb to over 100 Mb. Around 150 cases have been reported in the literature so far. Here, we report the unusual findings in three cases such as hepatomegaly, ataxia and females with mild intellectual disability that further expand the phenotypic spectrum of this disorder. This paper also stresses the need to perform microarray and/or multiplex ligation probe amplification in all cases of nonspecific intellectual disability.

A Single Case of Tourette's Syndrome Treated with Traditional Chinese Medicine.

The objective of this case study was to investigate the effectiveness of Chinese medicine in treating Tourette's syndrome. Tourette's syndrome is a childhood- onset disorder that is characterized by sudden, involuntary movements or tics. The participant in this study was a 33-year-old male who had been diagnosed with Tourette's syndrome at the age of 9 years. His major complaints included facial tics, shoulder shrugging, and clearing the throat. Using a combination of acupuncture, herbs, Gua-Sha, and lifestyle changes once a week for 35 treatments, all the symptoms were reduced by 70%, as reported by the patient. In this case, the results indicated that Chinese medicine was able to minimize the symptoms of Tourette's syndrome. Further investigation is needed to support this argument. Tourette's syndrome, which was first described in 1885 by a French physician named Gilles de la Tourette, is characterized by facial tics, involuntary body movements from the head to the extremities, or vocal tics, and it usually has its onset in childhood. It is a neuropsychiatric disorder. The treatment for Tourette's syndrome is based on pharmacological treatment, behavior treatment, and deep brain stimulation. Unfortunately, none of these could completely control the symptoms; furthermore, antipsychiatric drugs might cause additional side effects, such as Parkinson symptoms, tardive dyskinesia, and metabolic disturbances. Finding acupuncture and oriental medicine literature on treatment of Tourette's syndrome was difficult, especially that written in English. Some research papers that have been translated into English indicated that Chinese herbs and acupuncture could reduce the tics significantly. For example, a study by Dr Pao-Hua Lin reported the significant effects of using acupuncture and oriental medicine in treating 1000 Tourette's syndrome cases. This case was treated to further investigate the principles of Dr Lin's study.

Baseline Prevalence of Birth Defects Associated with Congenital Zika Virus Infection - Massachusetts, North Carolina, and Atlanta, Georgia, 2013-2014.

Zika virus infection during pregnancy can cause serious brain abnormalities, but the full range of adverse outcomes is unknown (1). To better understand the impact of birth defects resulting from Zika virus infection, the CDC surveillance case definition established in 2016 for birth defects potentially related to Zika virus infection* (2) was retrospectively applied to population-based birth defects surveillance data collected during 2013-2014 in three areas before the introduction of Zika virus (the pre-Zika years) into the World Health Organization's Region of the Americas (Americas) (3). These data, from Massachusetts (2013), North Carolina (2013), and Atlanta, Georgia (2013-2014), included 747 infants and fetuses with one or more of the birth defects meeting the case definition (pre-Zika prevalence = 2.86 per 1,000 live births). Brain abnormalities or microcephaly were the most frequently recorded (1.50 per 1,000), followed by neural tube defects and other early brain malformations(†) (0.88), eye abnormalities without mention of a brain abnormality (0.31), and other consequences of central nervous system (CNS) dysfunction without mention of brain or eye abnormalities (0.17). During January 15-September 22, 2016, the U.S. Zika Pregnancy Registry (USZPR) reported 26 infants and fetuses with these same defects among 442 completed pregnancies (58.8 per 1,000) born to mothers with laboratory evidence of possible Zika virus infection during pregnancy (2). Although the ascertainment methods differed, this finding was approximately 20 times higher than the proportion of one or more of the same birth defects among pregnancies during the pre-Zika years. These data demonstrate the importance of population-based surveillance for interpreting data about birth defects potentially related to Zika virus infection.

Clinical Features and Genetic Analysis of 48 Patients with Chronic Granulomatous Disease in a Single Center Study from Shanghai, China (2005-2015): New Studies and a Literature Review.

Chronic Granulomatous Disease (CGD) is a rare inherited primary immunodeficiency, which is characterized by recurrent infections due to defective phagocyte NADPH oxidase enzyme. Nowadays, little is known about Chinese CGD patients. Here we report 48 CGD patients in our single center study, which is the largest cohort study from Mainland China. The ratio of male to female was 11 : 1. The mean onset age was 0.29 years old, and 52% patients had an onset within the 1st month of life. The mean diagnosis age was 2.24 years old. 11 patients (23%) had died with an average age of 2.91 years old. 13 patients (28%) had positive family histories. The most prevalent infectious sites were the lungs (77%), followed by gastrointestinal tract (54%), lymph nodes (50%), and skin (46%). In addition, septicopyemia, thrush, and hepatosplenomegaly were also commonly observed, accounting for 23%, 23%, and 40% of the cases. Lesions due to BCG vaccination occurred in more than half of the patients. X-linked CGD due to CYBB gene mutations accounted for 75% of the cases, and 11 of them were novel mutations. Autosomal recessive inheritance accounted for 6% patients, including 1 patient with CYBA, 1 with NCF1, and 1 with NCF2 gene mutations.

Cardiopathies in children hospitalized at the University hospital Souro Sanou, Bobo-Dioulasso: echocardiographic and therapeutic aspects.

Children's heart diseases in Africa represent a public health problem that is difficult to cope with due to young population density, low socioeconomic status and lack of suitable technical platforms. This study aims to highlight the echocardiographic and therapeutic aspects of cardiopathies in children hospitalized at the pediatric department of the University Hospital Souro Sanou, Bobo-Dioulasso. We conducted a descriptive cross-sectional study over the period January 2013-December 2014 (24 months). It was based on a literature review of echocardiography reports performed on children under 15 years of age in the echocardiography laboratory of CHUSS and of medical records of their therapeutic follow-up. During the study period, 184 echocardiographic examinations were performed and allowed identification of 93 cases of children with heart disease (50.50% of cases). Among them, 71% (66/93) of cases with congenital cardiopathies were distinguished and 29% (27/93) of acquired cardiopathies. The most common congenital heart diseases were: IVC (27.2%), IAC (10.6%), AVC (7.5%), F4T (9.1%), CAT (6%), related forms ( 15%). Acquired heart diseases were dominated by rheumatic valve diseases (48%), hypokinetic dilated cardiomyopathy (33.3%) and pericardial tamponade (18.5%). Surgery was recommended in 53.7% (50/93) of cases, of whom 86% (43/50) with congenital heart diseases and 14% (7/50) with acquired heart diseases. 21% (9/43) of patients with congenital heart diseases underwent cardiac surgery. No acquired heart disease requiring surgery was surgically treated. Cardiopathies in children are frequent in Bobo-Dioulasso. Multidisciplinary strategies associated with resource optimization should improve the management of these cardiopathies.