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Ebstein’s anomaly was first described by Wilhelm Ebstein in 1866. At autopsy of a young man with a history of palpitations’ and dyspnoea followed—before his death—by cyanosis, cardiomegaly and systolic murmur, the Berlin based doctor described a tricuspid valve abnormality. This congenital malformation is characterised by any degree of inferior displacement of the proximal attachments of the septal and posterior leaflets of the tricuspid valve from the atrioventricular ring. It occurs in about 1–5 per 200 000 births and represents <1% of all congenital heart disease.1 Clinical presentation, treatments and outcome are varied and individual management is required.
MORPHOLOGY
Tricuspid valve anatomy
The rules of cardiac anatomy state that the valve belongs to the ventricle. The ventricle is divided into three components: the outlet, the apical trabecular and the inlet component which extends from the atrioventricular junction. During systole, the atrioventricular valve has to stay closed and resist the contraction of the ventricle to prevent backflow of blood. The tricuspid valve is attached to the atrioventricular junction of the right ventricle. The three leaflets are distinguished by their position: septal, anterior, and inferior leaflets. These leaflets are suspended from the muscular atrioventricular junction apart from a small area of membranous septum. They are joined by papillary muscles: the medial papillary muscle between the septal and anterior leaflets, the inferior between the septal and posterior leaflets, and the anterior between the posterior and the anterior leaflets.2
The embryologic origin of the tricuspid valve is the delamination from the ventricular myocardium at the atrioventricular junction. In Ebstein’s anomaly, the delamination, primarily of the septal and inferior leaflets, is incomplete. There are no cords to suspend the leaflets, so they arise from the cavity of the ventricle and are attached to its wall. An apical displacement of leaflets has been described, but a recent concept of rightward and anterior rotational displacement of the valve has also been considered. It is important to explain the special pattern of closure of the abnormal valve, with the right atrioventricular ring being in a horizontal position rather than a vertical position.3 Displacement results in “atrialisation” of the proximal portion of the right ventricle, a reduction in size of the functional right ventricle, and tricuspid regurgitation. The anterior leaflet is not usually displaced but is often redundant and curtain-like in the right ventricular cavity; it can cause right ventricular outflow obstruction. The distal attachment of this anterior leaflet affects the choice of surgical procedures. Anomalies in attachment of the leaflets and the displacement of the valvular orifices lie on a broad spectrum of clinical severity. Anterior and apical rotational displacement of the tricuspid valve and functional annulus directs it toward the right ventricular outflow tract and can cause obstruction of the right ventricular infundibulum.
Associated cardiac malformations
Others cardiac malformations are often associated with Ebstein’s anomaly, especially atrial septal defect. In utero, the reduced flow passing through the right ventricular outflow tract affects development of the pulmonary annulus, the main pulmonary arteries or its branches. It leads to an anatomic pulmonary atresia which differs from functional pulmonary atresia.
Abnormalities affecting prognosis are those in the left ventricle involving myocardium or valves in 39% of patients with Ebstein’s anomaly.4 In addition to the right sided heart anomaly, several studies reported systolic and, more recently, diastolic dysfunction of the left ventricle. The change in right ventricular geometry can lead to anomalies in the size, function and shape of the left ventricle, with paradoxical motion of the interventricular septum and regional contraction abnormalities. Moreover, left ventricular interstitial fibrosis caused by arterial oxygen desaturation and right ventricle overload, leading to left ventricular diastolic dysfunction, has been described in adults and neonates with Ebstein’s anomaly.5 6 Another intrinsic abnormality of left ventricular myocardium has recently been described: the left ventricular non-compaction. In affected patients, arrested myocardial morphogenesis results in excessively large trabeculations and intertrabecular recesses in the ventricular wall. This in turn can cause systolic and diastolic ventricular dysfunction, ventricular arrhythmias or systemic embolisation.7
Classification of Ebstein’s anomaly
In 1988, Carpentier and colleagues put forward an anatomic classification:
Type A: adherence of septal and posterior leaflets without restrictive volume of functional right ventricle
Type B: right ventricle atrialised with normal anterior leaflet
Type C: stenotic anterior leaflet
Type D: atrialisation of all the right ventricle except for a small infundibular component.
An echocardiographic grade of severity has been used in neonates. This Great Ormond Street score (Gose score) described by Celermajer involves calculating the ratio of the combined area of the right atrium and atrialised right ventricle to that of the functional right ventricle and left heart in a four chamber view at end-diastole. This ratio has four grades of increasing severity: ratio <0.5 = grade 1; ratio 0.5 to 0.99 = grade 2; ratio 1 to 1.49 = grade 3; and ratio >1.5 = grade 4. It provides information about prognosis: when the ratio is >1.5, 100% mortality is likely; a ratio of 1 to 1.4 indicates a decreased risk of death, with an early mortality of 10% but a mortality rate in early childhood of 45%; and a ratio <1 indicates 92% survival.6
ANTENATAL DIAGNOSIS
Ebstein’s anomaly is difficult to diagnose in the fetal period and carries a poor prognosis: the mortality rate is reported at 45% in utero.8 Most cases are sporadic and familial Ebstein’s anomaly is rare. Risk factors such as maternal therapeutic lithium intake have been recognised. In a fetus with Ebstein’s anomaly, the presentation is variable with possible features including cardiomegaly, right atrial enlargement, tricuspid valve regurgitation or dysplasia, arrhythmia or fetal hydrops. Tricuspid valve anomalies with tricuspid insufficiency should be investigated in the presence of right atrial enlargement. Two dimensional echocardiography with high resolution imaging in the fetus allows the description of tricuspid leaflet morphology as well as their proximal and distal attachments. Colour flow mapping and spectral Doppler imaging provide information related to the presence of associated tricuspid regurgitation and aid in the identification of any right ventricular outflow tract abnormality and pulmonary artery flow suggestive of pulmonary outflow obstruction or poor right ventricular function. In fetal life, functional and anatomical right ventricular outflow obstructions remain difficult to distinguish, and severe tricuspid regurgitation may simulate pulmonary atresia.8
The prognosis for the fetus diagnosed in utero with significant tricuspid valve disease is extremely poor, with a prenatal course that includes progressive right heart dilatation, with cardiac failure and lung hypoplasia in many, and the development of pulmonary stenosis or atresia later during the gestation of some. Survival rate is in the region of 49% at birth in fetuses with Ebstein’s anomaly or tricuspid valve dysplasia, but approximately 20% beyond 1 month of age.8 9 Factors indicating severity should be recognised. These include the Gose score, the absence of anterograde flow across the pulmonary valve due to severe tricuspid regurgitation, and in extreme cases fetal hydrops. Regular assessment of the evolution of the disease should be undertaken so that if the above signs appear, an elective delivery can be performed.9
CLINICAL FEATURES
A wide spectrum of symptoms has been described in patients with Ebstein’s anomaly, mainly due to haemodynamics effects such as tricuspid regurgitation, right to left shunt by atrial defect, and different degrees of right ventricular dysfunction. Right ventricular outflow tract obstruction can be associated with symptomatology of tricuspid valve malformation. Finally, symptoms are usually influenced by arrhythmias with accessory conduction pathways. Ebstein’s anomaly may present at any age, and variable clinical courses are correlated with the age of presentation.
Neonates
During the neonatal period, Ebstein’s anomaly leads mainly to haemodynamic symptomatology. Neonates may present with congestive heart failure (due to tricuspid regurgitation and right ventricular dysfunction) and cyanosis (tricuspid valve regurgitation increasing the right atrial pressure and resulting in a right to left shunt by way of an atrial septal defect or the usual neonate patent foramen ovale). Chest x ray helps in the diagnosis of an enormous cardiomegaly. Management during the first hours of life involves decreasing pulmonary vascular resistance in order to improve right ventricular function, thus reducing the overload. Then, to maintain a pulmonary flow, the ductus arteriosus should be made patent using prostaglandin treatment. However, a large patent ductus arteriosus maintains high pulmonary arterial pressure due to left to right shunt. Studies have reported a spiral haemodynamic failure in neonates with Ebstein’s anomaly.10 A “circular shunt” is maintained by a patent ductus arteriosus and causes an ineffective circulation of aortic blood back to the aorta without crossing the capillary bed. The blood leaving the aorta takes the route through a patent ductus, to the pulmonary arteries, to the right ventricle due to pulmonary insufficiency, to the right atrium through the dyplastic tricuspid valve, to the left atrium through the foramen ovale, then to the left ventricle whereupon it returns to the aorta (fig 1). Wald et al described the discontinuation of prostaglandin E1 treatment, which is often initiated for the first time in cyanotic neonatal diseases, in order to stop this spiralling deterioration. This approach reduced the mortality rate to 7%.10 Despite that, and the introduction of innovative surgical techniques, neonatal prognosis remains very poor. Before 1986 the mortality rates exceeded 80%, but from 1986 to 1996 these rates decreased to 47%.11 In a review, Celermajer found a survival of 67% at 1 year and 59% at 10 years. Such clinical or paraclinical features are predictive of mortality. Presence of cyanosis is associated with 50% mortality in the neonatal period and is 100% predictive of death when associated with a high Gose score; cardiomegaly >85% results in 100% mortality in neonates; and the presence of a large atrial septal defect, anatomic or functional pulmonary atresia and severe tricuspid regurgitation is invariably fatal.11
From childhood to adult
As soon as the neonatal and infant period is over, children reach adulthood developing right heart failure, and a new range of symptoms emerge. As neonates, severity of clinical signs depends on the degree of displacement of tricuspid valve leaflets. However, compared to the first month, the prognosis is better. The most common initial symptom is exercise intolerance. Patients with Ebstein’s anomaly are limited in their functional capacity to undertake exercise, with the occurrence of dyspnoea and cyanosis. Several factors play a part in decreasing a patient’s performance: tricuspid regurgitation, ventricular dysfunction, right-to-left shunt, and reduced cardiac output. Reduced exercise tolerance of non-operated patients with right-to-left shunt toward the atrial defect is correlated with oxygen saturation at rest. Surgical repair enables exercise capacity to be improved. With increasing age, adults with Ebstein’s anomaly present with arrhythmias, which, in those patients usually maintaining a patent foramen ovale, favour paradoxical embolisation, brain abscess and sudden death. In this population, the New York Heart Association (NYHA) score, the degree of cyanosis, and the presence or absence of tachyarrhythmias modulate the prognosis. Pregnancy is well tolerated in women with the mild form of Ebstein’s anomaly (without cardiomegaly, severe cyanosis or arrhythmias). Nevertheless, it remains associated with an increased risk of abortion, prematurity or congenital heart disease compared to the general population. Moreover, low birth weight is correlated significantly with the maternal degree of cyanosis. Paternal Ebstein’s anomaly increases the risk of congenital heart disease in the fetus.
CARDIAC IMAGING
Two dimensional echocardiography
Echocardiography is essential in order to identify the abnormal tricuspid valvar complex and to guide management. Two dimensional (2D) echocardiography can show inferior displacement of the proximal attachments of the tricuspid valve leaflets, distal attachments of the tricuspid valve leaflets, and right heart size and function (fig 2). In the apical four chamber view, the excessive apical displacement with regard to the hinge point allows the diagnosis of Ebstein’s anomaly. An apical displacement of the septal tricuspid valve leaflet from the insertion of the anterior mitral valve leaflets >8 mm/m2 body surface area distinguishes Ebstein’s malformation from other lesions with tricuspid regurgitation or right heart dilatation. This displacement into the right ventricular apex mainly involves the septal and posterior leaflets and so can be mistaken for right ventricular trabeculations. The apical four chamber view can also reveal pronounced atrial dilatation and enlargement of the atrialised right ventricle, defined by the wall separating the true from the functional annulus of the right ventricle. This dilatation is considered significant if the area of the right atrium combined with the atrialised right ventricle is larger than the area of the functional ventricle associated with the left atrium and left ventricle.6
Right ventricular dysplasia occurs in Ebstein’s anomaly with key diagnostic features, including wall thickening, dilatation and dyskinesis (defined as paradoxical septal motion, paradoxical systolic expansion of the atrialised right ventricle or significant decreased wall motion) of the atrialised or functional right ventricle. Paradoxical motion of the interventricular septum causes alteration in the right and left ventricular geometry and function. Non-geometric quantitative assessment of right and left ventricular function is measured by the reduction in size of the systolic cavity, calculating the area of the cavity in both systole and diastole and obtaining the fractional change in area, with a median normal value of 49%. A non-geometric myocardial performance index has been defined by the ratio of total time spent in isovolumic activity (contraction and relaxation) to the ejection time. An increasing myocardial performance index means ventricular systolic dysfunction.
The final feature of Ebstein’s anomaly is distal attachment of the tricuspid leaflets, and this affects both prognostic outcome and surgical management. The motion of the anterior leaflets is a positive prognostic factor for surgical outcome. In a normal heart, attachments are focal and connected to appropriate papillary muscles. In Ebstein’s anomaly, accessory attachments of the leaflets to the right ventricle wall (and sometimes linear attachments) are present. This tethering limits the motion of the leaflet and, even after repair, the tethered leaflets will not align properly with the septum or the other leaflets, causing significant residual regurgitation.
In tricuspid regurgitation with right sided heart enlargement, echocardiography remains the best technique to differentiate Ebstein’s anomaly from other tricuspid valve anomalies. Ebstein’s anomaly is the most common aetiology of primary tricuspid regurgitation and has to be differentiated from tethering of tricuspid valve leaflets by aberrant tendinous chords, which is one of the main differential diagnoses. Recently, Kobza and colleagues described this unknown mechanism of tricuspid regurgitation: an unusual chord inserted in the region of the tricuspid leaflets usually without chords. This anomaly, which may develop secondary to right ventricular or tricuspid annulus enlargement, does not match up with Ebstein’s anomaly criteria defined by the apical displacement of the septal leaflet >8 mm/m2 and the presence of elongated anterior leaflets.12
Three dimensional echocardiography and other imaging techniques
Precise description of the tricuspid anatomy by conventional 2D echocardiography remains difficult. Three dimensional (3D) echocardiography offers surface rendering views of the leaflet surface.13 Ebstein’s anomaly of the tricuspid valve consists of various degrees of inferior displacement of the proximal attachments of the septal leaflet. Since the apical four chamber plane provides good visualisation of the septal leaflet, 2D echocardiography enables the initial diagnosis of Ebstein’s anomaly. However, downward displacement could involve the anterior and posterior leaflets of the tricuspid valve. Precise description of the tricuspid anatomy could be difficult from the 2D planes only. The surface of the tricuspid leaflets, as well as the commissures, can be rendered by 3D echocardiography (fig 3). 3D echocardiography offers new ways to evaluate the ability and efficiency of surgical valve repair. Assessing Ebstein’s anomaly using real time 3D transthoracic echocardiography adds information about the leaflet surface and describes more precisely the tethering of the leaflets, and the right ventricular volume and function.
In patients with Ebstein’s anomaly, magnetic resonance imaging may provide pre- and postoperative information regarding ventricular function, muscle mass and valve morphology when echocardiography is inconclusive or technically not feasible.
ARRHYTHMIAS
In Ebstein’s anomaly, the right atrial enlargement, the frequent coexistence of an atrial septal defect and the high prevalence of accessory pathways predispose to the development of atrial tachyarrhythmias. If current surgical techniques, including tricuspid valve repair or replacement, are successful, the long term outcome is still affected by cardiac arrhythmias. The management of tachyarrhythmias remains a real challenge.
Electrocardiographic abnormalities
Classical electrocardiographic changes include tall and broad P waves, prolonged PR interval, and complete or incomplete right bundle branch block. Accessory atrioventricular pathways are revealed in 6–30% of patients with Ebstein’s anomaly, and can lead to both supraventricular and ventricular tachyarrhythmias. The most common supraventricular tachyarrhythmias are accessory pathway mediated reciprocating tachycardia, atrioventricular nodal re-entrant tachycardia, and atrial flutter or fibrillation. In the case of accessory pathways, ventricular pre-excitation is obvious in only 60% of patients with Ebstein’s anomaly and symptomatic tachyarrhythmias. In other patients without ventricular pre-excitation, the absence of right bundle branch block seems to be a strong predictor of accessory pathways, and becomes visible after radiofrequency catheter ablation. Tachyarrhythmias cause palpitation, syncope or sometimes sudden death and are more poorly tolerated, since haemodynamic and anatomic abnormalities are associated with Ebstein’s anomaly.
Accessory pathways are usually located on the right side or in the posteroseptal region. Electrophysiologic mapping and radiofrequency ablation remain difficult before tricuspid valve repair or replacement because of atrial dilatation, which disrupts anatomic landmarks and makes it difficult to find and target the atrioventricular junction with a catheter. Accessory pathways are often multiple, meet in a broad band, and abnormal endocardial activation potentials can often confound their identification.
Management
Ablation of accessory pathways has a lower success rate in patients with Ebstein’s anomaly than in those with anatomically normal hearts (76% compared to 95%), with a higher recurrence risk of 25%. Accessory pathway surgical ablation has resulted in excellent success rates without notable increases in early mortality (2.4%).14 Intraoperative electrophysiologic mapping is performed at normothermia before institution of cardiopulmonary bypass, followed by surgical ablation of the accessory pathways by transmural incision. These results are less impressive in cases of atrial flutter or fibrillation because of right atrial and intraoperative atrial incisions, which favour atrial arrhythmias with a recurrence rate of 25%, but which is reduced to 7% with adjunctive medical treatment. In a recent study, radiofrequency ablation of accessory pathways followed subsequently by surgical correction appeared to be less effective than a simultaneous approach with surgical ablation, even if prior catheter ablation reduced cardiopulmonary bypass time, complications and mortality.
SURGERY
Since the first report by Hunter and Lillehei, several different surgical techniques have been described to treat Ebstein’s anomaly: a tricuspid valve repair with biventricular repair, a univentricular repair with anastomosis, and a tricuspid valve replacement or transplant. If choosing the technique is difficult, so is deciding upon the timing of the surgery. In Ebstein’s anomaly, indications for surgery depend on the symptomatology, with cyanosis, arrhythmia, and the Carpentier anatomic classification all influencing the type of surgery. Neonatal features with high pulmonary pressure and Ebstein’s anomaly make surgery high risk with poor prognosis. In children, once infancy has passed, it is usual to wait for the clinical situation to deteriorate, with accentuation of the cyanosis and increased right ventricular dysfunction, before deciding to undertake surgery. However, to wait until these patients reach adulthood increases the risk of arrhythmias and ventricular dysfunction, with poor haemodynamic tolerance and adaptation following repair.
Tricuspid valve repair
Biventricular repair usually supports the use of anterior leaflets which often have no adherence with the underlying myocardium and are not displaced in the right ventricle. The tricuspid valve therefore becomes a one leaflet valve. Other procedures use displaced septal and posterior leaflets with some chordae tendineae and corresponding papillary muscle which are replaced from the native annulus and ventricular wall, respectively. Da Silva and colleagues reported a technique based on Carpentier’s concept: anterior and posterior leaflets are detached from their anomalous attachments and their free edges are rotated to be sutured to the septal edge of the anterior leaflet. This clockwise mobilisation forms a new tricuspid valve resembling a cone, resulting in less tricuspid regurgitation and fewer reoperations, and an improvement in the patient’s clinical status.15
A vertical or horizontal plication of the atrialised chamber is associated with this arrangement of the anterior leaflet, allowing a significant decrease in right ventricular size and so leading to a decompression of the left ventricle which improves its function, and provides more space for the lungs. To regain the right ventricular geometry completely, excision of the atrialised chamber has been proposed. But excision of the ventricular wall from apex to base increases the risk of arrhythmias and can lead to right coronary kinking. So the atrialised chamber may be left intact and incorporated into the functional right ventricle. Annuloplasty is undertaken by running a suture from the anteroposterior to the posteroseptal commissure, and may be reinforced by a prosthetic ring to avoid postoperative tricuspid regurgitation. The foramen ovale or atrial septal defect is closed. In case of severe right ventricular outflow tract obstruction or atresia, the repair is performed with a pericardial patch or with a pulmonary homograft.
Tricuspid valve replacement
When tricuspid valve repair is not feasible, tricuspid valve replacement is undertaken. A bioprosthetic as opposed to a mechanical valve is inserted in the anatomic tricuspid annulus, watching out for the atrioventricular node and the right coronary artery. The disadvantages of tricuspid replacement are anticoagulant complications associated with the mechanical valves or calcification and degeneration of bioprosthetic valves.
Cavopulmonary shunt
A bidirectional cavopulmonary shunt can be performed in association with biventricular repair in the case of right ventricular dysfunction. However, this shunt compromises subsequent access to the heart for catheter ablation of accessory pathways or placement of a pacemaker. In neonates, Fontan intervention is preferred to tricuspid valve replacement and can be selected when biventricular repair is not possible. Another surgical approach is the one-and-a-half ventricular repair, involving tricuspid valve repair with closure of the atrial septal defect and anastomosis of the superior vena cava only to the right pulmonary artery. This technique remains controversial, despite achieving low pressure in the inferior vena cava compartment, and producing results equivalent to the Fontan intervention.16
Ebstein’s anomaly of the tricuspid valve: key points
Ebstein’s anomaly is a malformation of the tricuspid valve, with apical displacement of the septal and posterior leaflets, resulting in a reduction in size of the right ventricle.
Clinical and paraclinical features change with age and severity of tricuspid valve anomaly, resulting in a wide spectrum of symptoms.
Two dimensional echocardiography is essential to identify the abnormal tricuspid valvar complex and to guide management.
Timing and the type of surgery are variable; surgical techniques range from biventricular or univentricular repair to tricuspid valve replacement.
Results of surgery
Recently, Dearani and colleagues published results on surgery for Ebstein’s anomaly over a 34 year period. In this retrospective study, repair was performed in 34% of 551 patients, including replacement in 64%, one-and-a-half ventricle repair in 2.9%, and Fontan intervention in 0.5%. Early mortality was 4.9%, which has decreased to 2.4% since 1999. With a follow-up of 7 years, the late death rate was 7.6%. Freedom from reoperation after replacement of the tricuspid valve compared with freedom from reoperation after valve repair was not significantly different (81.9% vs 83.1%, respectively).17
In young children, results show that Ebstein’s anomaly can be now repaired with a low mortality and low reoperation rate. In a population ranging in age from 5 months to 12 years at the time of operation, with a mean follow-up of 12.2 years, Danielson and colleagues reported actuarial survival at 5, 10, and 15 years of 92.3%, 89.9%, and 89.9%, respectively. Freedom from all reoperations at 5, 10, and 15 years was 91%, 76.9%, and 61.4%, respectively. Early mortality was 5.8% with higher risk if age was <2.5 years and weight was <10.7 kg.18 In contrast, tricuspid replacement (with mechanical or bioprosthetic valves) in young children leads to a high mortality (26%), especially in patients under 1 year old (64%), and is associated with complications including heart block requiring pacemaker (13%) and thrombosis (5%).
Clinical improvements are observed postoperatively: NYHA and heart size decrease, and exercise performance increases with a higher maximal consumption of oxygen, a lower ratio of minute ventilation to oxygen consumption, and higher rest and exercise blood oxygen saturation. If the right-to-left shunt influences preoperative performance, its disappearance following surgery plays a significant role in improvement of exercise tolerance, whatever the operative technique. Postoperatively, age, gender and heart size influence maximal oxygen uptake.
Surgery in neonates
Several techniques have been described, but successful repair has not been achieved and palliative procedures have poor outcomes. Remmsten et al recently reported a survival rate of 80% using right ventricular exclusion, including patching of the tricuspid valve and a modified Blalock–Taussig shunt.19 An innovative fenestration of the tricuspid valve patch was added to this procedure, first described by Starnes, allowing right ventricle decompression and avoiding right ventricle dilatation. The survivors have since undergone successful Fontan intervention. Right ventricle exclusion produces good results but requires at least two subsequent interventions after initial palliation. The single ventricle repair makes access difficult for catheter ablation of arrhythmias. Knott-Craig et al encouraged an early surgical management with biventricular repair and reported good results with 5 years’ follow up in three cases only.20 He described an innovative surgical technique in the first 3 weeks of life with subtotal closure of the atrial defect, extensive resection of the right atrium, and vertical plicature of the atrialised chamber.
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CONCLUSION
Ebstein’s anomaly is an uncommon malformation of the tricuspid valve and right ventricle, with an extremely variable natural history and prognosis with age. In neonates this right ventricular dysfunction can lead to a severe clinical situation with a poor prognosis. Symptomatology in young children and adults is essentially characterised by decreasing functional capacity and the occurrence of arrhythmias, which present a challenge for radiofrequency catheter ablation procedures. Diagnosis is achieved by echocardiography, which helps to evaluate the severity of the disease at the fetal stage. Nowadays, novel imaging techniques such as 3D echocardiography and magnetic resonance imaging enable precise anatomical and functional assessment of the tricuspid valves and the right ventricle.
Ebstein’s anomaly is initially described as a right heart disease but, with time, left heart abnormalities can contribute to symptomatology. Although it remains a disease with a poor prognosis, new anatomical and haemodynamic knowledge has led to improvements in the medico-surgical management of this condition.
REFERENCES
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Footnotes
Competing interests: In compliance with EBAC/EACCME guidelines, all authors participating in Education in Heart have disclosed potential conflicts of interest that might cause a bias in the article. The authors have no competing interests.