Acquired infundibular pulmonary stenosis associated with a congenital membranous ventricular septal defect (Gasul phenomenon) in a dog and discussion regarding causes of infundibular stenosis.

An aclinical Havanese dog was diagnosed with a membranous restrictive ventricular septal defect. The patient was represented later in their natural history due to the development of syncope. At that time the patient was diagnosed with acquired pulmonary infundibular stenosis. Balloon dilation of the stenosis was performed successfully twice over the patient's lifetime. The patient died suddenly approximately 14 months after the second balloon dilation. A discussion regarding primary infundibular pulmonary stenosis versus causes of acquired infundibular pulmonary stenosis including anomalous muscle bundles (double chamber right ventricle), tetralogy of Fallot, and infundibular stenosis is presented.

Double-orifice mitral valve repair.

We present the case of a 6-year-old patient with double-orifice mitral valve and severe regurgitation, which was successfully repaired. We demonstrate that mitral valve repair may be attempted even in complex anatomy such as the double-orifice mitral valve, where it can offer excellent results.

Aterosclerosis calcificada del tronco de la arteria pulmonar, estenosis de las arterias pulmonares principales y dilatación postestenótica de arterias pulmonares segmentarias en un paciente con síndrome de Alagille / Calcified atherosclerosis of the pulmonary trunk, stenosis of the main pulmonary arteries, and post-stenotic dilation of segmental pulmonary arteries in a patient with Alagille syndrome

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A classical case of the Gasul phenomenon.

This case demonstrates the development of secondary infundibular stenosis in a 10-year-old male child with documented large non-restrictive perimembranous ventricular septal defect in infancy - the classical Gasul phenomenon.

20 years of arterial switch operation for simple TGA.

BACKGROUND: Arterial switch operation became the golden treatment for simple transposition of the great arteries (sTGA). We describe our experience with the arterial switch operation regarding long-term outcome and the need for re-intervention. Nevertheless, supravalvular pulmonary stenosis (SPS) remains a concern in the long run. We assess the evolution of SPS over time and evaluate the effect of technical modifications on SPS during our experience. METHODS: We performed a retrospective study on 133 patients operated with ASO for TGA between October 1991 and November 2009. Last report method was used. We reviewed our pediatric cardiology and cardiac surgery database to examine the echocardiographic data and electrocardiograms. A mean follow-up of 9.2 years (+/- 5.83 SD) was reached. RESULTS: One (0.8%) patient deceased postoperatively due to cardiogenic shock. The overall actuarial freedom from reoperation (open and percutaneous) was 88.1%, 78.5% and 76.9% at 1, 5 and 10 years. SPS needed to be treated in 17 patients. Valve regurgitation at final investigation was maximal moderate in 5 patients for the aortic valve, 10 for pulmonary valve and 3 in tricuspid valve. CONCLUSIONS: ASO shows excellent long-term results in sTGA with a very low morbidity and mortality and is therefore the procedure of choice. Re-intervention rate is determined by SPS. Since the extensive mobilization of the pulmonary arteries and the creation of a longer neo-pulmonary root, reduction in SPS was seen with no re-interventions in the second half of the group. To obtain a final comparison with the atrial switch operation, a longer Follow-up is necessary.

Tetralogía de Fallot en el paciente adulto: a propósito de un caso / Fallots tetralogy in an adult patient: Apropos of a case

La tetralogía de Fallot constituye el 10 % de todas las cardiopatías congénitas. Representa la cardiopatía congénita cianógena más frecuente en el adulto, con una incidencia de 5,8 por ciento al nacer, pero solo el 6 por ciento de los pacientes no operados viven a los 30 años de edad y el 3 por ciento a los 40 años. Después de realizada una historia clínica completa de la paciente y los exámenes complementarios necesarios, se procede a revisar la literatura y se comparan los hallazgos y en especial la evolución con lo descrito por otros autores. Se presenta a la paciente MDCC de 48 años de edad con historia clínica No 18605 que ingresa el 21/2/2013 por falta de aire intensa de 4 días de evolución, que tendía a incrementarse al realizar esfuerzo físico o al caminar largas distancia, que no se aliviaba al cambio de posición. Describe, además, que ese mismo día comenzó a notarse como si se le inflamaran haran los pies, sin coloración cianótica, ni se acompañaba de tos, expectoración, ni fiebre. Examen físico, hallazgos positivos. Aparato cardiovascular: ruidos cardiacos rítmicos de buen tono e intensidad, se ausculta soplo sistólico pulmonar III/VI, que se irradia hacia el resto del área precordial. Se le realiza la analítica de laboratorio y los exámenes de imágenes disponibles (Rx de tórax, Tomografía Axila Computarizada contrastada de tórax, ecocardiograma doppler). Se analizan fisiopatológicamente los síntomas de la pacientes y su supervivencia con tan buena calidad de vida (incluyendo dos embarazos, uno de ellos a término) sin haber sido operada y sin un seguimiento adecuado. Aunque es baja la supervivencia de los pacientes con tetralogía de Fallot después de los 40 años algunos como la paciente estudiada lo logra y con buena calidad de vida, esto no significa que no pueda presentar ciertas enfermedades, como insuficiencia cardiaca derecha o congestiva, arritmias y endocarditis(AU)

Fallot's tetralogy amounts to 10 % of all the congenital cardiopathies. It is the most frequent cyanogenic congenital cardiopathy in adults, with a 5,8 percent incidence at birth, but only 6 percent of the non-operated patient lives at the age of 30 years, and 3 percent at the age of 40. After making the clinical record of the female patient and the necessary complementary examination, we reviewed the literature and compared the findings, specially the evolution, with the facts described by other authors. We present the MDCC patient, aged 48 years with clinical record Nr 18605, entering the service on February 21st, 2013, for lack of breath of 4 days of evolution, with a tendency to increase when exercising or when walking long distances, and that do not alleviate when changing position. We also describe that the same day we began to notice feet swelling, without a cyanotic coloration or cough, expectoration or fever accompaniment. Physical examination showed positive findings. Cardiovascular tract: rhythmic cardiac noise of good tonus and intensity; we auscultated a systolic pulmonary blow III/VI, irradiating to the rest of the pre cordial area. Laboratory analysis and available imaging examination (Thoracic Rx, thoracic contrasted computerized tomography, Doppler echocardiogram) were carried out. We physio-pathologically analyzed the patient's symptoms and her survival (including two pregnancy, one of them a term pregnancy) without being operated and without an adequate follow. Although the survival of the patients with Fallot¡'s tetralogy after they are 40 years old is low, some of them, like the studied patient, achieve it and with good life quality, it does not mean that they might not present several diseases, like right or congestive cardiac insufficiency, arrhythmia and endocarditis(AU)

Isolated subpulmonic fibrous ring, mirror-image dextrocardia and situs solitus in a young lady unreported and a near miss.

Congenital diseases causing obstruction of the right ventricular outflow tract (RVOT) are common, but the isolated subpulmonary membrane/ring is extremely rare and can be difficult to diagnose precisely, especially in adults. We report a case of surgically resected isolated subpulmonic fibrous ring in a lady with mirror-image dextrocardia and abdominal situs solitus that was misdiagnosed by echocardiography as a subaortic membrane.

Development of supravalvular pulmonary artery stenosis following a Nuss procedure.

We report a case of a 13-year old girl with pectus excavatum who had a Nuss procedure and two years later a new cardiac murmur appeared which on investigation was diagnosed as supravalvular pulmonary artery stenosis. Following removal of the Nuss bar the stenosis resolved.

LEOPARD syndrome.

LEOPARD syndrome (LS) is a rare hereditary disorder, characterised mainly by skin, facial and cardiac abnormalities. We report on the case of a six-year-old Djiboutian with typical features of LS. Multiple cardiovascular problems are described, including pulmonary infundibular, valvular and supra-valvular stenosis. A favourable course was observed after successful cardiac surgery. This is the first reported case of LS from the horn of Africa.

Primary infundibular stenosis and pedigree analysis in three Golden Retriever littermates.

Three eight-week-old Golden Retriever puppy littermates were evaluated because of left basilar systolic murmurs and were diagnosed with primary infundibular stenosis. Pedigree analysis in this line was also performed to identify a mode of inheritance. All dogs were asymptomatic at the time of diagnosis; two of the three had congenital lesions in addition to primary infundibular stenosis. Two additional affected dogs were identified in the line, and pedigree analysis suggested an autosomal recessive mode of inheritance. Another, unrelated golden retriever was also identified with isolated infundibular stenosis in the record database. Primary infundibular stenosis should be considered in the differential diagnoses for golden retriever dogs with a left basilar systolic murmur, and is often associated with complex congenital cardiac disease. Primary infundibular stenosis may worsen in severity with time, and in this line of dogs an autosomal recessive pattern of inheritance is likely.

Imaging congenital heart disease in adults.

Transthoracic echocardiography is the first-line modality for cardiovascular imaging in adults with congenital heart disease (ACHD). The windows of access that are possible with transthoracic echocardiography are, however, rarely adequate for all regions of interest. The choice of further imaging depends on the clinical questions that remain to be addressed. The strengths of MRI include comprehensive access and coverage, providing imaging of all parts of the right ventricle, the pulmonary arteries, pulmonary veins and aorta. Cine images and velocity maps are acquired in specifically aligned planes, with stacks of cines or dynamic contrast angiography providing more comprehensive coverage. Tissues can be characterised if necessary, and MRI provides relatively accurate measurements of biventricular function and volume flow. These parameters are important in the assessment and follow-up of adults after repairs for tetralogy of Fallot or transposition of the great arteries and after Fontan operations. The superior spatial resolution and rapid acquisition of CT are invaluable in selected situations, including the visualisation of anomalous coronary or aortopulmonary collateral arteries, the assessment of luminal patency after stenting and imaging in patients with pacemakers. Ionising radiation is, however, a concern in younger patients who may need repeated investigation. Adults with relatively complex conditions should ideally be imaged in a specialist ACHD centre, where dedicated echocardiographic and cardiovascular MRI services are a necessary facility. General radiologists should be aware of the nature and pathophysiology of congenital heart disease, and should be alert for previously undiagnosed cases presenting in adulthood, including cases of atrial septal defect, aortic coarctation, patent ductus arteriosus, double-chambered right ventricle and congenitally corrected transposition.

Surgical correction of congenital heart disease at 76 years of age.

The management of adult congenital heart disease is challenging and poses specific problems. We report a patient with ventricular septal defect and pulmonary stenosis who underwent successful repair and coronary artery bypass grafting at the age of 76 years.

Respiratory distress after surgery of RVOT pathologies: a word of caution on pseudoaneurysm development.

Pseudoaneurysm of the right ventricle outflow tract (RVOT) is a rare complication in pediatric cardiac surgery. We report a patient who developed a right ventricular pseudoaneurysm 8 months after RVOT enlargement using a pericardial patch for infundibular pulmonary stenosis. Our patient was born with severe pulmonary valvular stenosis and treated with percutaneous balloon valvotomy in the neonatal period. Six months later, she developed infundibular pulmonary stenosis, which required surgical resection of right ventricle infundibular trabeculations and bovine pericardial patch enlargement. The postoperative period was normal. She was readmitted to hospital 5 months later complaining of wheezing, coughing and shortness of breath. Echocardiography showed a huge aneurysmal dilatation of the outflow patch in connection with the right ventricular cavity. The patient underwent resection of the pseudoaneurysm and former patch, followed by interposition of a bovine jugular vein conduit between the RVOT and pulmonary bifurcation. The early postoperative period was uncomplicated. On echocardiography, no significant residual gradient was measured through the conduit and there was no insufficiency of the valve. RVOT reconstruction with patch enlargement, homograft or conduit implantation can be the origin of pseudoaneurysms. Although their incidence is rare, they are often asymptomatic before becoming quite large and causing compression symptoms as in our patient with respiratory complaints due to airway compression. It is important to follow up these patients closely, especially in the first year after surgery since most aneurysms develop within 6 months of surgery.