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  • Abstract: Large ventricular septal defect x
  • Abstract: Patent ductus arteriosus x
  • Abstract: Pulmonary atresia with intact ventricular septum x
  • Abstract: Pulmonary atresia with ventricular septal defect x
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Open access

EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Echocardiographic assessment of transposition of the great arteries and congenitally corrected transposition of the great arteries

Meryl S Cohen and Luc L Mertens

Echocardiographic assessment of patients with transposition of the great arteries and congenitally corrected transposition requires awareness of the morphology and commonly associated lesions. The pre-operative echocardiography should include a full segmental and sequential analysis. Post-operative assessment is not possible without awareness of the type of surgical procedure performed and consists of assessing surgical connections and residual lesions.

Open access

EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Tetralogy of Fallot: diagnosis to long-term follow-up

R Bedair and X Iriart

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect, affecting 3 in 10,000 live births. Surgical correction in early childhood is associated with good outcomes, but lifelong follow-up is necessary to identify the long-term sequelae that may occur. This article will cover the diagnosis of TOF in childhood, the objectives of surveillance through adulthood and the value of multi-modality imaging in identifying and guiding timely surgical and percutaneous interventions.

Open access

Quadruple valve replacement in a patient with severe rheumatic heart disease

Isaac Adembesa, Adriaan Myburgh, and Justiaan Swanevelder


We present a patient with rheumatic heart disease involving all the heart valves. An intraoperative transoesophageal echocardiography confirmed severe mitral stenosis, severe aortic regurgitation, severe tricuspid regurgitation and stenosis, and severe pulmonary stenosis. The patient underwent successful quadruple valve replacement during a single operation at the Groote Schuur Hospital, Cape Town, South Africa.

Learning points:

  • Rheumatic heart disease can affect all the heart valves including the pulmonary valve.

  • Intraoperative transoesophageal echocardiography is key for diagnosis, monitoring and confirmation of successful surgical result during heart valve surgery.

  • Combined surgical procedure of all four valves is possible though associated with long procedural time.

Open access

Exercise stress echocardiography in patients with valvular heart disease

Vishal Sharma, David E Newby, Ralph A H Stewart, Mildred Lee, Ruvin Gabriel, Niels Van Pelt, and Andrew J Kerr

Stress echocardiography is recommended for the assessment of asymptomatic patients with severe valvular heart disease (VHD) when there is discrepancy between symptoms and resting markers of severity. The aim of this study is to determine the prognostic value of exercise stress echocardiography in patients with common valve lesions. One hundred and fifteen patients with VHD (aortic stenosis (n=28); aortic regurgitation (n=35); mitral regurgitation, (n=26); mitral stenosis (n=26)), and age- and sex-matched controls (n=39) with normal ejection fraction underwent exercise stress echocardiography. The primary endpoint was a composite of death or hospitalization for heart failure. Asymptomatic VHD patients had lower exercise capacity than controls and 37% of patients achieved <85% of their predicted metabolic equivalents (METS). There were three deaths and four hospital admissions, and 24 patients underwent surgery during follow-up. An abnormal stress echocardiogram (METS <5, blood pressure rise <20 mmHg, or pulmonary artery pressure post exercise >60 mmHg) was associated with an increased risk of death or hospital admission (14% vs 1%, P<0.0001). The assessment of contractile reserve did not offer additional predictive value. In conclusion, an abnormal stress echocardiogram is associated with death and hospitalization with heart failure at 2 years. Stress echocardiography should be considered as part of the routine follow-up of all asymptomatic patients with VHD.

Open access

Echocardiographic RV-E/e′ for predicting right atrial pressure: a review

A J Fletcher, S Robinson, and B S Rana

Right atrial pressure (RAP) is a key cardiac parameter of diagnostic and prognostic significance, yet current two-dimensional echocardiographic methods are inadequate for the accurate estimation of this haemodynamic marker. Right-heart trans-tricuspid Doppler and tissue Doppler echocardiographic techniques can be combined to calculate the right ventricular (RV) E/e′ ratio – a reflection of RV filling pressure which is a surrogate of RAP. A systematic search was undertaken which found seventeen articles that compared invasively measured RAP with RV-E/e′ estimated RAP. Results commonly concerned pulmonary hypertension or advanced heart failure/transplantation populations. Reported receiver operating characteristic analyses showed reasonable diagnostic ability of RV-E/e′ for estimating RAP in patients with coronary artery disease and RV systolic dysfunction. The diagnostic ability of RV-E/e′ was generally poor in studies of paediatrics, heart failure and mitral stenosis, whilst results were equivocal in other diseases. Bland–Altman analyses showed good accuracy but poor precision of RV-E/e′ for estimating RAP, but were limited by only being reported in seven out of seventeen articles. This suggests that RV-E/e′ may be useful at a population level but not at an individual level for clinical decision making. Very little evidence was found about how atrial fibrillation may affect the estimation of RAP from RV-E/e′, nor about the independent prognostic ability of RV-E/e′ . Recommended areas for future research concerning RV-E/e′ include; non-sinus rhythm, valvular heart disease, short and long term prognostic ability, and validation over a wide range of RAP.

Open access

EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Congenital left-sided heart obstruction

Michelle Carr, Stephanie Curtis, and Jan Marek

Congenital obstruction of the left ventricular outflow tract remains a significant problem and multilevel obstruction can often coexist. Obstruction can take several morphological forms and may involve the subvalvar, valvar or supravalvar portion of the aortic valve complex. Congenital valvar stenosis presenting in the neonatal period represents a spectrum of disorders ranging from the hypoplastic left heart syndrome to almost normal hearts. Treatment options vary dependent on the severity of the left ventricular outflow tract obstruction (LVOTO) and the variable degree of left ventricular hypoplasia as well as the associated lesions such as arch hypoplasia and coarctation.

Open access

An atypical case of pulmonary embolism from a jugular vein

A E Velcea, S Mihaila Baldea, D Muraru, L P Badano, and D Vinereanu


Neck venous malformations and their potentially life-threatening complications are rarely reported in the available literature. Cases of aneurysmal or hypo-plastic jugular vein thrombosis associated with systemic embolization have not been frequently reported. We present the case of a 60-year-old male, without any known risk factors for thromboembolic disease, admitted for sudden onset dyspnea. The physical examination was remarkable for a right lateral cervical mass, expanding with Valsalva maneuver. Thoracic CT with contrast established the diagnosis of bilateral pulmonary embolism and raised the suspicion of superior vena cava and right atrial thrombosis. Bedside transthoracic echocardiography confirmed the presence of a large right atrial thrombus, with intermittent protrusion through the tricuspid valve. Systemic thrombolysis with Alteplase was initiated shortly after diagnosis, in parallel with unfractionated heparin, with complete resolution of the intracavitary thrombus documented by echocardiography. The patient showed significant improvement in symptoms and was later started on oral anticoagulation. Computed vascular tomography of the neck was performed before discharge, showing hypoplasia of the left internal jugular vein and aneurismal dilation of the contralateral internal jugular vein, without thrombosis. There were no identifiable systemic causes for thrombosis. Surgical resection of the aneurismal jugular vein was excluded, because of its potential to cause intracranial hypertension. The preferred therapeutic option in this case was long-term oral anticoagulation.

Learning points:

  • Internal jugular venous malformations, such as aneurisms or hypoplasia, could be associated with an increased risk of thrombosis and major embolic events.

  • Systemic thrombolysis can be an efficient solution in cases of pulmonary embolism with right heart thrombosis.

  • Multimodality imaging is greatly valuable in clarifying the diagnosis of atypical cases.

Open access

EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Echocardiographic assessment of left to right shunts: atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent arterial duct

Antigoni Deri and Kate English

This review article will guide the reader through the basics of echocardiographic assessment of congenital left to right shunts in both paediatric and adult age groups. After reading this article, the reader will understand the pathology and clinical presentation of atrial septal defects (ASDs), ventricular septal defects (VSDs), atrioventricular septal defects (AVSDs) and patent arterial duct. Echocardiography is the mainstay in diagnosis and follow-up assessment of patients with congenital heart disease. This article will therefore describe the echocardiographic appearances of each lesion, and point the reader towards specific features to look for echocardiographically.

Open access

Successful percutaneous closure of spiral atrial septal defect

Mashail Alobaidan, A Saleem, H Abdo, and J Simpson


The case report of a 15-year-old patient with an unusual form of atrial septal defect is described. Echocardiography showed separation of the secundum and primum atrial septums due to abnormal posterior and leftward attachment of the primum septum into the roof of the left atrium. The morphology has been variably described as a ‘double’ atrial septum or ‘spiral’ atrial septal defect. Despite the technical challenge of this form of atrial septal defect, it was effectively closed by ensuring that all relevant septal structures were incorporated between the discs of the occlusion device. This was associated with a stable position and good medium-term outcome. This contrasts with the experience of others where device embolisation or technical failure has been described.

Learning points

  • The spiral atrial septal defect is characterised by an apparently ‘double’ atrial septum.

  • Such atrial septal defects (ASDs) have been associated with a high rate of technical failure of transcatheter closure.

  • 3D echocardiography assists in understanding the anatomy of the defect.

  • Following deployment of the ASD occlusion device transoesophageal echocardiography is essential to ensure that both septum primum and secundum are between the occluder discs.

  • Catheter closure can be successful if close attention is paid to the morphology of the defect and incorporation of margins within the discs of the septal occluder.

Open access

Echocardiographic approach to catheter closure of atrial septal defects: patient selection, procedural guidance and post-procedural checks

Alexandra Savis and John Simpson

Echocardiography is the imaging modality of choice to diagnose different types of atrial septal defect and to determine which defects are suitable for catheter occlusion. In addition to assessment of defect size and rims, transoesophageal echocardiography may be used to guide the procedure itself including device placement, procedural complications and post-procedural checks. This review covers a practical approach to this subject and is accompanied by online videos illustrating the technique.