Quadricuspid aortic valve with ruptured sinus of valsalva aneurysm to the right atrium.

A 32-year-old man with a sudden onset of chest pain and progressive dyspnea was found to have a ruptured sinus of Valsalva aneurysm to the right atrium with an associated quadricuspid aortic valve. Echocardiographic and angiographic images are presented, with real time transthoracic 3D echo. The patient was successfully operated.

Factors predicting the progress of mitral valve disease in surgically treated adults with ostium primum atrial septal defects.

OBJECTIVE: This study was undertaken to analyze the clinical profile, associated features, and surgical treatments of adults operated on for ostium primum atrial septal defects, particularly factors influencing progression of mitral valve disease. METHODS: We retrospectively studied all patients aged 18 years and older operated on at our institution with reference to patient clinical features, investigation findings, surgical records, and outpatient follow-up data. RESULTS: Fifty-one patients, 29 female and 22 male, underwent operation at a mean age of 27.3 years (SD 6.9). Of these, 80% were in New York Heart Association functional class I or II, with a most frequent presenting symptom of dyspnea. On echocardiography, 88% had cleft mitral valve, 35% had moderate mitral regurgitation, and 4% had severe mitral regurgitation. According to echocardiography and available cardiac catheterization data, 27% had moderate pulmonary arterial hypertension and 8% had severe. In-hospital mortality was 1.9%. At mean follow-up of 36 months, 94% of patients were in functional class I. Mitral regurgitation was moderate in 21% and severe in 8%, with 1 patient undergoing mitral valve replacement. Factors associated with increased risk of moderate or severe mitral regurgitation on follow-up were preoperative moderate or severe pulmonary arterial hypertension (P = .008) and female sex (P = .009). CONCLUSION: Surgical correction of ostium primum atrial septal defects in adults can be undertaken successfully with low mortality and excellent symptomatic results. Regular follow-up is required to assess progression of mitral regurgitation, which is more likely in women and those with preoperative pulmonary arterial hypertension.

Concurrent diagnosis of rheumatic mitral stenosis & aortoarteritis: a rare combination.

A 21-year-old lady was initially diagnosed with rheumatic mitral stenosis and then 18 months later with asymptomatic aortoarteritis. She was treated with percutaneous interventions for both problems. This is a very rare combination of conditions, which has previously been reported in a few autopsy specimens. We highlight the importance of thorough echocardiography in the patient and also suggest how future cases could be treated at one catheterisation.

Aortopulmonary window in adults: diagnosis and treatment of late-presenting patients.

OBJECTIVES: Aortopulmonary window is an uncommon condition, particularly so in adulthood because it is usually fatal in infancy or childhood if untreated. Very few cases of those who have survived to adulthood and been operated on successfully have been described. Our study aimed to provide clinical, investigative, surgical, and outcome details of such patients. DESIGN AND SETTING: Retrospective study of consecutive adult patients with aortopulmonary window treated at a tertiary charitable cardiovascular institute in South India between 1996 and 2006. RESULTS: Six adult patients successfully underwent aortopulmonary window closure. Five of the six patients had been correctly diagnosed on echocardiography, while one was only diagnosed after cardiac catheterization for unexplained pulmonary arterial hypertension. Four of the patients had large defects with severe pulmonary arterial hypertension, with pulmonary vascular resistance index (PVRI) ranging from 5.2 to 15.9 at baseline. All showed significant reversibility with oxygen administration, with PVRI on oxygen falling to between 0.6 and 2.2. These patients successfully underwent cardiopulmonary bypass. The other two patients with small lesions underwent ligation off-pump. There was no early or late mortality among these patients. All were in New York Heart Association class I, on follow-up ranging from 3 months to 8 years. CONCLUSIONS: Aortopulmonary window may rarely present in adulthood. The diagnosis can usually be made by careful echocardiography alone. Even in the presence of severe pulmonary arterial hypertension, if a significant reversibility in pulmonary vascular resistance can be demonstrated with oxygen, the condition can be successfully corrected with good long-term outcomes.

Mixed supra- and infradiaphragmatic totally anomalous pulmonary venous connection in an adolescent.

An unusual type of mixed totally anomalous pulmonary venous drainage in an 11-year-old boy was diagnosed on angiography. The left-side veins drained via a vertical vein into the portal venous system, while the right-side veins drained high into the superior vena cava. Surgical correction of this abnormality was successfully performed.

Giant ruptured sinus of Valsalva aneurysm: diagnosis on echocardiography.

A giant ruptured sinus of Valsalva aneurysm was diagnosed on transthoracic and subsequent transesophageal echocardiography, in a 45-year-old man who presented with gradual onset shortness of breath. Although the initial presentation was insidious, he later rapidly deteriorated. We discuss the unusual clinical course in a patient with such a large aneurysm and discuss the likely reasons.

Clinical presentation and investigation findings in cardiac myxomas: new insights from the developing world.

BACKGROUND: Cardiac myxomas are an uncommon condition and most of the available information on their clinical features comes from smaller series of patients from developed countries. Our aim was to quantify and correlate the clinical and investigation findings in cardiac myxomas in a developing country and compare them with existing data. METHODS: A retrospective study of case notes, electrocardiograms, and x-rays of 171 patients treated for cardiac myxoma from February 1992 to October 2006 at a large charitable institution in South India was conducted. Frequency of different clinical findings and relationships between these findings, age, sex, tumor location, and size were calculated. RESULTS: The mean age at presentation was 37.1 years. Dyspnea was the most common symptom. Embolism was found in 9% of patients and systemic symptoms in 20% of patients. Auscultation abnormalities were present in 89% of patients, including a tumor plop in 50%. Left atrial enlargement was the most common electrocardiographic abnormality (35%), whereas cardiomegaly was the most common chest x-ray finding (55%). Raised erythrocyte sedimentation rate was found in 75% and anemia in 45% of patients. Female patients and patients with right atrial myxomas more commonly had systemic symptoms. Tumor size correlated with electrocardiographic and x-ray abnormalities. CONCLUSIONS: Cardiac myxomas present at a younger age in developing countries. Almost all patients were symptomatic because they presented for medical care at an advanced stage of disease. Certain clinical manifestations depend on site and size of the tumor as well as age of the patient. A high index of suspicion is necessary for making an early diagnosis.

Imaging features of aorto-right atrial tunnel: a report of two cases.

Aorto-right atrial tunnel is a rare congenital anomaly with very few cases described in the literature. It has been classified into anterior and posterior types and we present two operated cases, one of each type. The radiologic, echocardiographic, and angiographic features of the condition are highlighted.