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.

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.

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.

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...

Oculofaciocardiodental syndrome: a rare case and review of the literature.

Oculofaciocardiodental syndrome is a rare genetic disorder affecting ocular, facial, dental, and cardiac systems. The clinical diagnosis of oculofaciocardiodental syndrome can be challenging due to a wide variety of symptoms. Oculofaciocardiodental syndrome is found only in females due to its X-linked inheritance pattern and embryonic lethality for males. Radiculomegaly of canines is the most consistent finding in these patients. In this report we present a female patient with characteristic facial features, as well as a comprehensive overview of oculofaciocardiodental syndrome. Diagnosis of oculofaciocardiodental syndrome in this patient was verified by genetic analysis, during which we found a novel mutation in BCOR.

The percutaneous closure of a large pseudoaneurysm of the ascending aorta with an atrial septal defect Amplatzer occluder: two-year follow-up.

Pseudoaneurysm of the ascending aorta is a high-risk complication following cardiac surgery. The present report describes excellent two-year follow-up results after the percutaneous closure of a very large pseudoaneurysm with an Amplatzer atrial septal defect occluder. The original cavity in the anterior mediastinum with maximal diameter 15 cm remained as only a small scar. The patient was without serious health problems both early and after two years.

Combined treatment for multiple cardiac defects with interventional techniques.

Multiple congenital cardiac defects are usually addressed by cardiac surgery. We present our experience with simultaneous transcatheter treatment of multiple defects in children. Ten children, six females and four males, with multiple defects underwent treatment with interventional technique. The mean age was 4.4 +/- 2.6 years (range, 7 months to 8 years). The cardiac diagnosis was patent ductus arteriosus (PDA) and valvular pulmonary stenosis (PS) in two, atrial septal defect (ASD) and PDA in two, ASD and PS in two, PDA and aortic stenosis (AS) in three (severe left ventricular dysfunction in two), and perimembranous ventricular septal defect (VSD) and valvular PS in one. The ASDs were closed with an Amplatzer septal occluder (mean size, 16 +/- 4 mm), four PDAs were closed with an Amplatzer duct occluder, and three with a Cook's detectable coil. Mean balloon size used to dilate the pulmonary valve was 18 +/- 4 mm, and for the aortic valve this was 12 +/- 2 mm. There was a 70% (+/-15%) postprocedure reduction of gradients across the stenotic valves. The closure rate was 75% for PDAs in the catheterization laboratory, 80% for ASDs, and there was a mild residual intradevice leak in the VSD. In conclusion, interventional technology addressing multiple congenital cardiac defects as a combined procedure in the catheterization laboratory is safe and effective.

Use of rapid prototyping in the care of patients with structural heart disease.

Advances in surgery, interventional techniques, and critical care have allowed more than 90% of children born with structural heart defects to survive into adulthood. In addition, advances in imaging technology continue to raise awareness of hemodynamically significant intracardiac shunt lesions in both adults and children. Adult cardiologists are now faced with the daunting task of caring for patients with complex structural heart lesions, a population subset that at one time was exclusively cared for by pediatric cardiologists and congenital heart disease specialists. Given the wide range of anatomic complexity present in patients with structural heart disease, the definition and anatomic clarification of their structural abnormalities through high-quality noninvasive imaging has become paramount. Current two-dimensional imaging techniques, however, remain limited in their ability to effectively illustrate the complex three-dimensional relationships present in structural heart disease. Rapid prototyping, a process by which three-dimensional digital surface models are converted into physical models, represents the next evolution in advanced image processing and may serve as a means to improve our understanding of the many forms of structural heart disease. Ultimately, the technology may be used to enhance the level of care provided to the growing number of patients with structural heart defects. We recently reviewed the novel cardiovascular application of rapid prototyping. This review examines the expanded applications of rapid prototyping in the care and treatment of adult patients with structural heart disease.

Septal defects: surgeons do it better.

Until the recent availability of percutaneous devices, the role of the septal defect occluder was played by surgeons. In this paper the pros and cons of the two techniques in the approach to the atrial and ventricular septal defects are analyzed. Although the reported results are rather interesting, it seems clear that one of the crucial aspects to guarantee good results is the selection of patients for the transcatheter approach. Surgery is not confined by the constraints of defect anatomy, and good results may be achieved without regard to patient selection or an otherwise unfavorable anatomy of the defect. Up to now, the follow-up of transcatheter closure has been relatively short and cannot be compared with that of surgery. Therefore, caution should be exercised when proposing the transcatheter approach to septal defects as the true alternative to conventional surgery.

Management algorithm in pulmonary atresia with intact ventricular septum.

Pulmonary atresia with intact ventricular septum (PAIVS) is a disease with remarkable morphologic variability, affecting not only the pulmonary valve but also the tricuspid valve, the RV cavity and coronary arteries. With advances in interventional techniques and congenital heart surgery, the management of PAIVS continues to evolve. This review is an attempt at providing a practical approach to the management of this disease. The basis of our approach is morphologic classification as derived from echocardiography and angiography. Group A, patients with good sized RV and membranous atresia, the primary procedure at presentation is radiofrequency (RF) valvotomy. Often it is the only procedure required in this group with the most favourable outcome. Patients with severely hypoplastic RV (Group C) are managed along the lines of hearts with single ventricle physiology. The treatment at presentation is patent ductus arteriosus (PDA) stenting with balloon atrial septostomy or conventional modified Blalock Taussig (BT) shunt. Bidirectional Glenn shunt may be done 6-12 months later followed by Fontan completion after a suitable interval. Patients in Group B, the intermediate group, are those with borderline RV size, usually with attenuated trabecular component but well developed infundibulum. The treatment at presentation is RF valvotomy and PDA stenting +/- balloon atrial septostomy. Surgical re-interventions are not uncommonly required viz. bidirectional Glenn shunt when the RV fails to grow adequately (11/2 - ventricle repair) and right ventricular outflow tract (RVOT) reconstruction for subvalvar obstruction or small pulmonary annulus. Catheter based interventions viz. repeat balloon dilatation or device closure of patent foramen ovale (PFO) may also be required in some patients.

Assessment of patient radiation doses during transcatheter closure of ventricular and atrial septal defects with Amplatzer devices.

The purpose of this study was to estimate the radiation dose to which children are exposed during cardiac catheterizations for the treatment of ventricular and atrial septal defects. Radiation doses were estimated for 46 children aged 1-18 years. These children were treated for secundum atrial septal defects (ASD group) for perimembranous ventricular septal defects (VSD group) or underwent a routine diagnostic catheterization (diagnostic group). Thermoluminescent dosimeters (TLDs) were attached in locations, representing the lateral entrance dose, the posterior entrance dose, the thyroid dose, and the gonad dose, respectively. A dose area product (DAP) meter was also attached externally on the posterior-anterior (PA) tube to give a direct value in cGy cm(2) for each procedure. The patient's entrance dose from the PA field ranged from 1.5 to 185.0 mGy for all patients, while the lateral entrance dose varied from 0.9 to 204 mGy. Radiation exposure to the thyroid and the gonads was found to vary from 0.4 to 8.3 and 0.1 to 2.1 mGy, respectively. The DAP meter recorded DAP values for the posterior tube, between 46 and 3,700 cGy cm(2). The mean effective dose was found to be 7.7, 16.2, and 33.3 mSv for the diagnostic, the ASD, and the VSD group, respectively. Very strong correlation was found between the DAP values and the entrance radiation dose measured with TLDs. The mean entrance dose received from therapeutic cardiac catheterizations using the Amplatzer devices was found approximately twice the dose received from a diagnostic one. Even for the most complex procedures, the maximum entrance dose was at least 10 times lower than the threshold, associated with skin erythema.

Intracardiac echocardiography.

This article describes currently available intracardiac ultrasound (ICE) technology contrasting it with intravascular ultrasound (IVUS) highlighting their differences. Clinical applications in the electrophysiologic and cardiac catheterization laboratory are discussed and current limitations addressed. Intracardiac echocardiography (ICE) is possible because lower frequency transducers (in contrast to higher frequency IVUS devices) have been miniaturized and mounted onto catheters capable of percutaneous insertion into the heart. These lower frequency transducers are capable of enhanced tissue penetration, permitting high-resolution 2D "whole heart" imaging. Also, with the introduction of the newest phased array transducer, Doppler hemodynamic data in addition to high resolution imaging can also be obtained. ICE facilitates electrophysiologic procedures by guiding transseptal catheterization, enabling endocardial anatomy visualization and targeting of arrhythmogenic substrate, ensuring optimal ablation electrode/tissue contact and promptly diagnosing procedural complications. Promising non-electrophysiologic applications include guidance of percutaneous closure of septal defects, percutaneous mitral balloon valvuloplasty and complex cardiac biopsy. Current limitations include monoplanar imaging, narrow field of view, and relatively large size of the catheter. Intra-cardiac imaging is now a clinical tool and has the potential to play an important role in diagnostic and therapeutic interventional procedures. Further refinement and miniaturization of these transducers, through continued technological progress, will make way for primary operator controlled, integrated ultrasound-guided interventional devices.

High intensity focused ultrasound effect on cardiac tissues: potential for clinical application.

High intensity focused ultrasound (HIFU) is an evolving technology with potential therapeutic applications. Utilizing frequencies of 500 kHz to 10 MHz, HIFU causes localized hyperthermia at predictable depths without injuring intervening tissue. Applications in neurosurgery, urology, oncology and, more recently, cardiology for selective cardiac conduction tissue ablation have been promising. A 'noninvasive' technique for causing localized tissue damage to relieve hemodynamic and life-threatening obstruction in patients with congenital cardiac anomalies could replace more invasive procedures. We, therefore, investigated the ability of HIFU to create lesions in mammalian cardiac tissues ex vivo. Porcine valve leaflet, canine pericardium, human newborn atrial septum, and right atrial appendage were studied. Specimens were mounted and immersed in a water bath at room temperature. Using a 1-MHz phased array transducer, ultrasound energy was applied with an acoustic intensity of 1630 W/cm(2) or 2547 W/cm(2) until a visible defect was created (duration 3 to 25 sec). Macroscopic and microscopic examination demonstrated precise defects ranging from 3 to 4 mm in diameter. No damage was identified to the surrounding tissues. Our study concluded that HIFU can create precise defects in different cardiac tissue without damage to the surrounding tissue. Further investigation is needed to assess potential clinical uses of this technology.

Interventional procedures for congenital heart disease.

Interventional techniques available for use in treating congenital heart disease include balloon dilation of valves and vessels, stent placement and coil embolization of collaterals, patent ducts and other arterial fistulae. In addition, a variety of devices for closure of atrial and ventricular septal defects and patent ducts currently are under investigation. Radiofrequency ablation of arrhythmias also is applicable to the pediatric population.