Clinical Presentation and Therapy of Atrioventricular Septal Defect.

Atrioventricular septal defects (AVSDs) consist of a number of cardiac malformations that result from abnormal development of the endocardial cushions. AVSDs occur in 0.19 of 1000 live births and constitute 4-5 % of congenital heart defects. AVSDs can be categorized as incomplete (or partial) or complete, and intermediate or transitional.

Are We Forgetting About IgA? A Re-examination of Coronavirus Disease 2019 Convalescent Plasma.

BACKGROUND: While convalescent plasma (CP) may benefit patients with COVID-19, fundamental questions remain regarding its efficacy, including the components of CP that may contribute to its therapeutic effect. Most current serological evaluation of CP relies on examination of total immunoglobulin or IgG-specific anti-SARS-CoV-2 antibody levels. However, IgA antibodies, which also circulate and are secreted along the respiratory mucosa, represent a relatively uncharacterized component of CP. STUDY DESIGN AND METHODS: Residual samples from patients and CP donors were assessed for IgM, IgG, and IgA anti-SARS-CoV-2 antibody titers against the receptor-binding domain responsible for viral entry. Symptom onset was obtained by chart review. RESULTS: Increased IgA anti-SARS-CoV-2 antibody levels correlated with clinical improvement and viral clearance in an infant with COVID-19, prompting a broader examination of IgA levels among CP donors and hospitalized patients. Significant heterogeneity in IgA levels was observed among CP donors, which correlated weakly with IgG levels or the results of a commonly employed serological test. Unlike IgG and IgM, IgA levels were also more likely to be variable in hospitalized patients and this variability persisted in some patients >14 days following symptom onset. IgA levels were also less likely to be sustained than IgG levels following subsequent CP donation. CONCLUSIONS: IgA levels can be very heterogenous among CP donors and hospitalized patients and do not necessarily correlate with commonly employed testing platforms. Examining isotype levels in CP and COVID-19 patients may allow for a tailored approach when seeking to fill specific gaps in humoral immunity.

A case of a complete atrioventricular canal defect in a ferret.

BACKGROUND: Atrioventricular canal defect is a rare congenital disorder of the heart and describes the presence of an atrial septal defect, a variable presentation of ventricular septal alterations including ventricular septal defect malformations in the mitral and tricuspid valves. The defect has been described in human beings, dogs, cats, pigs, and horses. CASE PRESENTATION: This paper describes the case of a complete atrioventricular canal defect in a four-year-old intact male pet ferret (Mustela putorius furo), which was presented due to posterior weakness, ataxia, and decreased appetite. A loud systolic murmur, dyspnea, and hind limb paraparesis were detected during the clinical examination. Thoracic radiographs showed generalized cardiomegaly and lung edema. ECG showed sinus rhythm with prolonged P waves and QRS complexes. Echocardiography showed a large atrial septal defect, atrioventricular dysplasia, and a ventricular septal defect. Palliative treatment with oxygen, furosemide, spironolactone, enalapril, diltiazem, and supportive care was chosen as the therapy of choice. The ferret recovered gradually during hospitalization. A follow-up examination at three and six months showed stabilization of cardiac function. CONCLUSIONS: To the authors knowledge, this is the first time an atrioventricular canal defect has been described in a pet ferret.

Short to medium term outcomes of right ventricular outflow tract stenting as initial palliation for symptomatic infants with complete atrioventricular septal defect with associated tetralogy of Fallot.

OBJECTIVES: To assess the impact of right ventricular outflow tract (RVOT) stenting as the primary palliation in infants with complete atrioventricular septal defect with associated tetralogy of Fallot (cAVSD/TOF). BACKGROUND: Historically, palliation of symptomatic patients with cAVSD/TOF has been achieved through surgical systemic to pulmonary artery shunting. More recently RVOT stenting has evolved as an acceptable alternative in patients with tetralogy of Fallot. METHODS: Retrospective review of all patients with cAVSD/TOF who underwent RVOT stenting as palliation over a 13-year period from two large tertiary referral centers. RESULTS: Twenty-six patients underwent RVOT stenting at a median age of 57 days (interquartile range [IQR] 25.5-106.5). Median weight for stent deployment was 3.7 kg (IQR 2.91-5.5 kg). RVOT stenting improved oxygen saturations from a median of 72% (IQR 70-76%) to 90% (IQR 84-92%), p < .001. There was a significant increase in the median Z-score for both branch pulmonary arteries at median follow-up of 255 days (IQR 60-455). Eight patients required RVOT stent balloon dilatations and 8 patients required re-stenting for progressive desaturation. The median duration between reinterventions was 122 days (IQR 53-294 days). Four patients died during the follow-up period. No deaths resulted from the initial intervention. To date, definitive surgical intervention was achieved in 19 patients (biventricular repair n = 15) at a median age of 369 days (IQR 223-546 days). CONCLUSION: RVOT stenting in cAVSD/TOF is a safe and effective palliative procedure in symptomatic infants, promoting pulmonary artery growth and improving oxygen saturations.

Twiddler's syndrome with occult lead insulation damage in a child with epicardial pacemaker leads.

We present a rare case of incidentally diagnosed Twiddler's syndrome in a child 7 years after implantation of a dual-chamber pacemaker system with epicardial leads. During revision, an insulation defect of the ventricular lead was evident, despite unremarkable prior pacemaker lead testing. The lead was repaired and a new generator was suture-fixated to prevent re-occurrence of generator manipulation.

Estimation of intracardiac shunts in young children with a novel indicator dilution technology.

Clinical evaluation of intracardiac shunts in children is not straightforward. Echocardiography can only diagnose the presence of a shunt but does not estimate the shunt ratio. This can be a critical factor that influences treatment options. In this single-center, prospective, observational, method-comparison study, we validate the ability of a novel monitoring device COstatus to estimate the intracardiac shunt ratio (Qp/Qs) of pulmonary (Qp) to systemic (Qs) blood flow in young children before and after corrective cardiac surgery. The indicator dilution technology COstatus monitor was compared to two other more invasive reference techniques, perivascular ultrasonic flow probes (placed around the pulmonary truncus and ascending aorta) and the oximetric shunt equation (using arterial and venous blood gases). Our study revealed that the COstatus monitor detected intracardiac shunts with high sensitivity and specificity but there was some underestimation of the shunt ratios compared to the reference techniques.

Oculo-Facio-Cardio-Dental Syndrome: A Case Report about a Rare Pathological Condition.

(1) Background: Oculo-facio-cardio-dental (OFCD) syndrome is a rare pathological condition with an X-linked dominant trait that only occurs in females; no males are born with OFCD syndrome. This syndrome is characterized by congenital cataracts with secondary glaucoma ocular defects, ventricular and atrial septal defects, or mitral valve prolapses. Facial traits are a long narrow face and a high nasal bridge with a bifid nasal tip. Dental anomalies include radiculomegaly, oligodontia, root dilacerations, malocclusion, and delayed eruption. (2) Methods: This clinical report describes a 26-year-old girl who suffers from OFCD syndrome and who was treated with a multidisciplinary approach. The treatment plan included orthodontic treatment, orthognathic surgery, namely LeFort I and a Bilateral Sagittal Split Osteotomy, and occlusal rehabilitation with implants. (3) Discussion: Early diagnosis and multidisciplinary treatment of orthodontic, orthognathic surgery and occlusal rehabilitation with implants make it possible to maintain tooth function and improve aesthetics with good prognoses for success. In this paper, we report a case of a female patient with OFCD syndrome, who was referred for orthodontic treatment and occlusal rehabilitation and treated with a multidisciplinary approach.

Unilateral lung agenesis, hiatal hernia and atrioventricular septal defect: a rare combination of congenital anomalies.

Unilateral lung agenesis is a relatively rare congenital anomaly with a reported incidence of 1 in 15 000 births. It is frequently associated with other congenital malformations. Some of the sequelae of lung agenesis are potentially life-threatening. Here, we report a case of left lung agenesis in association with hiatal hernia and atrioventricular septal defect, a rare combination of anomalies which have not been described previously in the literature.

Assessing the borderline ventricle in a term infant: combining imaging and physiology to establish the right course.

PURPOSE OF REVIEW: The purpose of this review is to describe the challenges associated with the diagnosis and treatment of children with borderline ventricles. A borderline ventricle is one in which there is concern that it will not be able to support its circulation. If a biventricular repair is attempted and fails, outcome is often poor. Thus, this early decision is important. RECENT FINDINGS: For the borderline right ventricle, options to add an additional source of pulmonary blood flow make the surgical strategy a bit more flexible than for patients with a borderline left ventricle. In general, outcome for a so-called one and one-half ventricle repair are generally good, though the long-term outcome and the effects of this physiology on lifelong exercise performance and quality of life remain to be seen. For the small left ventricle, often multiple surgeries are required to 'force' blood into the left ventricle and potentially help it grow. Though this strategy is successful in some, in others it results in significant residual cardiac issues including pulmonary hypertension. SUMMARY: Determining whether a patient will be better off in the long term with a marginal biventricular repair versus a Fontan circulation remains one of the most difficult problems in the field of pediatric cardiology and cardiac surgery.

Aortoseptal angle and pressure gradient reduction following balloon valvuloplasty in dogs with severe subaortic stenosis.

INTRODUCTION: To determine the relationship between aortoseptal angle (AoSA) and the short- and long-term systolic pressure gradient (PG) reduction following combined cutting and high-pressure balloon valvuloplasty (CB/HPBV) in dogs with severe subaortic stenosis. ANIMALS: Retrospective study of 22 client-owned dogs of various breeds with severe subaortic stenosis (mean left ventricular to aortic PG = 143 mmHg; range = 80-322 mmHg) that underwent CB/HPBV. MATERIALS AND METHODS: Initial angiographic and left apical and right-sided parasternal long-axis view echocardiographic video loops were used for measuring the angle between the plane of the interventricular septum and the longitudinal axis of the ascending aorta. The PG reduction ratio immediately after CB/HPBV and 6 and 12 months later were compared with AoSA. RESULTS: Weak correlations were observed for all instances of PG reduction ratio and AoSA type. Significantly greater mean differences of PG reduction ratio were observed for angles >160° than for angles <160° at 24 h (>160° mean: 54.45, standard error [SE]: ±3.8; <160° mean: 39.88, SE: ±2.09), 6 months (>160° mean: 57.73, SE: ±10.9; <160° mean: 28.22, SE: ±3.42), and 12 months (>160° mean: 76.11, SE: ±17.5; <160° mean: 27.61, SE: ±6.44; p=0.003). CONCLUSIONS: Dogs with AoSA >160° on right-sided parasternal long-axis view echocardiograms responded with a greater PG reduction following CB/HPBV than did dogs with AoSA <160°. This suggests that AoSA is associated with long-term outcomes of CB/HPBV, and measurement could help in the evaluation of dogs that are candidates for CB/HPBV.

Pulmonary valvuloplasty for pulmonary atresia-restrictive ventricular septal defect.

Pulmonary atresia with restrictive ventricular septal defect is a rare congenital cardiac anomaly. A Blalock-Taussig shunt and surgical perforation of the atretic pulmonary valve is often performed as the initial palliation. We present our experience of utilizing both transesophageal and epicardial echocardiography during surgical pulmonary valvuloplasty in a 22-day-old neonate with pulmonary atresia with restrictive ventricular septal defect. The atretic pulmonary valve was perforated using a sheath introduced through the pulmonary artery.

Down Syndrome with Complete Atrioventricular Septal Defect, Hypertrophic Cardiomyopathy, and Pulmonary Vein Stenosis.

The prevalence of congenital heart disease in infants with Down syndrome is 40%, compared with 0.3% in children who have normal chromosomes. Atrioventricular and ventricular septal defects are often associated with chromosomal aberrations, such as in trisomy 21, whereas hypertrophic cardiomyopathy is chiefly thought to be secondary to specific gene mutations. We found only one reported case of congenital hypertrophic cardiomyopathy and atrioventricular septal defect in an infant with Down syndrome. Here, we report atrioventricular septal defect, hypertrophic cardiomyopathy, and pulmonary vein stenosis in a neonate with Down syndrome-an apparently unique combination. In addition, we discuss the relevant medical literature.

Oclusão percutânea de comunicação interatrial ou de forame oval guiada por ecocardiograma intracardíaco / Percutaneous closure of atrial septal defect or foramen ovale guided by intracardiac echocardiography

O ecocardiograma transesofágico (ECO-TE) é o método mais utilizado para guiar otratamento percutâneo da comunicação interatrial (CIA) e do forame oval (FOP), mas a necessidade de um outro profissional para realizá-lo e de anestesia geral constituem inconvenientes para seu emprego. Oecocardiograma intracardíaco (ECO-IC) apresenta-se como alternativa ao ECO-TE, pois pode ser realizado pelo próprio operador e demanda apenas anestesia local, com leve ou nenhuma sedação. Nosso objetivo foi relatar a experiência do serviço com a oclusão de CIA/FOP guiada por ECO-IC. Métodos: O ECO-IC utiliza cateter de ultrassom, que é introduzido por via venosa em câmaras cardíacas direitas e, por meio de posicionamento variável do transdutor, obtém as imagens adequadas para a intervenção. Foram avaliadas as taxas de sucesso do procedimento e as complicações. Resultados: De 2011 a 2015, foram realizados 201 procedimentos guiados pelo ECO-IC, sendo 139 empacientes com CIA e 62 com FOP. A maioria dos pacientes era do sexo feminino (64,2%), as idades variaramde 7 a 78 anos (36,6 ± 19,3 anos) e o peso variou de 28 a 92 kg (62,5 ± 13,0 kg). Foram utilizadas próteses Occlutech Figulla®, e todas as intervenções tiveram sucesso, com tempos de fluoroscopia de 5,7 ± 2,4 minutos e tempo de procedimento de 21,5 ± 6,4 minutos. Dois pacientes (2,0%) apresentaram taquicardia supraventricular transitória e outros dois pacientes evoluíram com fístula arteriovenosa na via de acesso, com resolução espontânea no primeiro mês. Conclusões: O ECO-IC forneceu informações anatômicas precisas para guiar o fechamento da CIA/FOP com sucesso e eliminou as principais desvantagens do ECO-TE...

Transesophageal echocardiography (TEE) is the most widely used method to guide the percutaneous treatment of atrial septal defect (ASD) and patent foramen ovale (PFO), but the necessity of another professional to perform it and the need for general anesthesia are potential disadvantages. Intracardiac echocardiography (ICE) is seen as an alternative to TEE, as it can be performed by the interventionist and requires only local anesthesia with mild or no sedation. The aim of this study was to report our experience with ASD/PFO occlusion guided by ICE. Methods: The ICE uses an ultrasound catheter, which is intravenously inserted in the right heart chambers and acquires images for the intervention through variable positioning of the transducer. Success and complication rates of the procedure were evaluated. Results: From 2011 to 2015, 201 procedures guided by ICE were performed, comprising 139 in patients with ASD and 62 in those with PFO. Most patients were female (64.2%), ages ranged from 7 to 78 years (36.6 ±19.3 years), and weight ranged from 28 to 92 kg (62.5 ± 13.0 kg). Occlutech Figulla® prostheses were used and all interventions were successful, with fluoroscopy time of 5.7 ± 2.4 minutes and procedure time of 21.5 ± 6.4 minutes. Two patients (2.0%) had transient supraventricular tachycardia and two others had arteriovenous fistula at the access site, with spontaneous resolution in the first month of follow-up. Conclusions: ICE provided accurate anatomical information to guide the closure of the ASD/PFO and successfully eliminated the main drawbacks of TEE...

Procedimentos percutâneos combinados em defeitos estruturais e congênitos / Combined percutaneous treatment of structural and congenital heart defects

Defeitos congênitos múltiplos são tradicionalmente corrigidos cirurgicamente, mas, atualmente, podem ser tratados percutaneamente. Existem poucos relatos na literatura atestandosua eficácia e segurança. Objetivamos descrever uma experiência com a realização de procedimentoscombinados para tratar diferentes defeitos, congênitos e estruturais, numa mesma sessão terapêutica. Métodos: Desde 2007, foram tratados, numa mesma sessão terapêutica, diferentes defeitos. Todos foram selecionados por ecocardiograma. Os procedimentos foram realizados segundo as técnicas tradicionais já descritas para cada defeito encontrado. Resultados: Foram tratados dez pacientes, cinco do sexo masculino, com idades de 1 a 67 anos, e pesos de 11 a 90 kg. O defeito mais prevalente de forma isolada foi a persistência do canal arterial (PCA, n = 5), seguido da comunicação interatrial ostium secundum (CIA OS, n = 4) e da comunicação interventricular (CIV, n = 4). As combinações mais frequentes foram CIV com PCA (n = 2) e CIV com CIA OS (n = 2). Foram dilatadas duasestenoses valvares pulmonares com CIA OS e com forame oval patente (FOP), e uma coarctação de aorta com PCA. Adicionalmente, foi ocluído um apêndice atrial esquerdo com FOP e foi embolizada uma fístula aortopulmonar com PCA. Todos os procedimentos foram bem-sucedidos. O tempo médio de seguimento foi de 31 ± 28,1 meses, havendo apenas duas complicações. Não houve nenhum óbito. Conclusões: A pequena série de casos relatada mostrou que os procedimentos combinados foram seguros e eficazes, podendo ser reproduzidos por operadores experientes em centros especializados, podendo vir a se constituir como primeira opção terapêutica para esses pacientes...

Multiple congenital defects are traditionally corrected surgically, but nowadays can be treatedpercutaneously. There are few reports in the literature attesting to its efficacy and safety. We aimed to describe an experience with combined procedures to treat different congenital and structural defects, in a single therapeutic session.Methods: Since 2007, different defects were treated in a single treatment session. All were selected byechocardiography. The procedures were performed using traditional techniques already described for each defect.Results: Ten patients were treated, five males, aged 1-67 years, weighting 11-90 kilograms. The most prevalent isolated defect was patent ductus arteriosus (PDA, n = 5), followed by ostium secundum atrial septal defects (ASD, n = 4) and ventricular septal defects (VSD, n = 4). The most common combinations were VSD with PDA (n = 2) and VSD with osASD (n = 2). Two pulmonary valve stenosis were dilated with ASD and patent foramen ovale (PFO), and one aorta coarctation with PDA. Additionally, a left atrial appendage with PFO was occluded and an aorto pulmonary fistula with PDA was embolized. All procedures were successful. The mean follow-up was 31 ± 28.1 months, with only two complications. There were no deaths.Conclusions: The small number of reported cases showed that the combined procedures were safe andeffective and can be reproduced by experienced operators in specialized centers and may be considered asthe first therapeutic option in these patients...

Intervencionismo en las cardiopatías congénitas del adulto / Percutaneous intervention in adult congenital heart disease

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