Pediatric Cor Triatriatum


Background

Cor triatriatum is a rare congenital cardiac anomaly in which a fibromuscular membrane divides the atrium in two. In its most common form, cor triatriatum sinister, the left atrium is divided into an upper chamber that receives the pulmonary veins and a lower chamber that is related to the left atrial appendage and the mitral valve orifice. [1]

The location of the atrial appendage is a key landmark in this congenital malformation. It differentiates cor triatriatum from a physiologically similar condition, supravalvular mitral stenosis. In cor triatriatum the left atrial appendage is invariably associated with the lower chamber, which is below the membrane.

Rarely, a fibromuscular membrane that is a remnant of the right valve of the sinus venosus divides the right atrium; this finding, so-called cor triatriatum dexter, is usually asymptomatic and is mostly reported as an incidental finding. [2However, fetal right ventricular hypoplasia has been described associated with this anomaly and has been hypothesized to result from abnormal fetal circulation leading to growth restriction of the fetal right ventricle. [3Owing to its clinical relevance, the remainder of this chapter focuses on cor triatriatum sinister.

Cor triatriatum represents 0.1% of all congenital cardiac malformations and may be associated with other cardiac defects in as many as 50% of cases. Examples of associated cardiac defects include atrial septal defect, persistent left superior vena cava with an unroofed coronary sinus, partial anomalous pulmonary venous connectionventricular septal defect, and more complex cardiac lesions, such as tetralogy of Fallot, atrioventricular canal, and double outlet right ventricle. Associated bicuspid pulmonary valve, aortic valve atresia, and heterotaxy have also been described. [4]

In classic cor triatriatum, the pulmonary venous chamber receives all pulmonary veins and it drains into the left atrium through a variable-sized orifice. The natural history of this defect depends on the size of the communicating orifice between the upper and lower atrial chambers. If the communicating orifice is small, the patient presents critically ill and may succumb at a young age (usually during infancy) to congestive heart failure and pulmonary edema. If the connection is larger, patients may present in childhood or young adulthood with a clinical picture similar to that of mitral stenosis. Cor triatriatum may also be an incidental finding when it is nonobstructive.

Other anatomical variants of cor triatriatum include a pulmonary venous chamber that receives part of the pulmonary veins and communicates with the left atrium, and a pulmonary venous chamber receiving all or part of the pulmonary veins that does not connect with the left atrium.

Congenital pulmonary vein stenosis is a very rare association with cor triatriatum. [5Variable types of subtotal cor triatriatum may be noted, with only the right or left pulmonary veins draining into the upper chamber. [6]

Pathophysiology

Initially, the fetal lungs and pulmonary veins are connected to the systemic venous circulation. Subsequently, a dorsal outgrowth from the common atria, also referred as common pulmonary vein, evaginates and joins the pulmonary veins, whereas the connection to the systemic circulation disappears. As the fetal heart grows, the common pulmonary vein is completely absorbed. Failure of this dorsal outgrowth to join the pulmonary veins results in total anomalous pulmonary venous drainage (TAPVD). An abnormal connection between the common pulmonary vein and the atria results in any of the variants of cor triatriatum.

The critical anatomic feature of cor triatriatum is a diaphragm that divides the left atrium into 2 chambers (see the videos below).

Long-axis parasternal view demonstrating a left atrial membrane separating pulmonary vein inflow from left ventricular (mitral valve) inflow. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Long-axis parasternal view depicting 2D image of cor triatriatum sinister membrane and color Doppler of pulmonary venous flow through the orifice of the membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Short-axis parasternal view depicting right and left pulmonary vein flow proximal to the cor triatriatum left atrial membrane and left atrial appendage orifice distal to the cor triatriatum left atrial membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Subxiphoid coronal image of the posterior left atrial chamber that receives pulmonary venous flow separated from the rest of the left atrium by the cor triatriatum membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Apical 5-chamber view demonstrating a 4-5 mm left atrial membrane orifice with mild pulmonary venous inflow restriction. Note the presence of an associated perimembranous ventricular septal defect (VSD) with tricuspid septal aneurysmal tissue. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.

It consists of fibromuscular tissue, and the proximal chamber that is created represents a vestigial common pulmonary vein. The pulmonary veins thus drain into the proximal chamber.

Outcome depends on the size of the communication to the distal chamber, which communicates with the mitral valve. If present, a patent fossa ovalis or secundum atrial septal defect permits decompression of the proximal chamber into the right atrium.

The presentation of cor triatriatum is one of decreased cardiac output and pulmonary venous hypertension. If a connection between the common pulmonary venous chamber and the right atrium is present, pulmonary overcirculation may result in significant right ventricular enlargement.

Epidemiology

Frequency

United States

This is a very rare malformation. Incidence is approximately 0.1-0.4% of all infants with congenital cardiac disease.

Mortality/Morbidity

Approximately 75% of patients die in infancy (generally from pulmonary hypertension) if the defect is unrepaired. If the communication between the proximal and distal chambers is not restrictive or if an atrial septal defect allows decompression of the hypertensive left atrium, the prognosis is significantly improved.

The only treatment is surgical correction. Most postoperative deaths occur in the first 30 days. The early mortality rate in each large series was consistently 15-20%. Early deaths had a higher rate of associated severe cardiac anomalies.

Long-term results are excellent, with long term survival of 80-90% in patients surviving surgery. Survivors have excellent functional results without residual sequelae and a life expectancy that approaches that of the general population. This is particularly true when repair is performed in infancy.

Complications

If the condition is unrecognized and untreated, problems include pulmonary edema, right heart failure, and death.

Late complications include recurrence of the obstructive membrane with recurrence of symptoms. [5Similar symptoms may occur later on in life if the membrane is incompletely excised.

Ischemic stroke as a late complication from undiagnosed cor triatriatum (sinister) in an adult has been described. [7]

Race

No race predilection is reported.

Sex

A slight male predilection is observed, with a male-to-female ratio of 1.4:1.

Age

Diagnosis is primarily made in infancy. Later presentation does occur but is usually in childhood or early adulthood; in this patient population, 85% are younger than 40 years. Rarely, patients presenting in their eighth or ninth decade of life have been reported.

CLINICAL PRESENTATION


History

Cor triatriatum is essentially a form of left atrial inflow obstruction and presents with signs and symptoms of pulmonary venous obstruction.

Most patients present during infancy with a restrictive opening in the membrane. These infants usually present with evidence of low cardiac output, including pallor, diminished peripheral pulses, and tachypnea.

Feeding difficulties, poor weight gain, and respiratory distress are common.

Presentation later in life is less classic; however, when the patient becomes symptomatic, evidence of pulmonary venous obstruction predominates. In these patients, the membrane may become calcified with its orifice becoming smaller or the patient may develop mitral valve insufficiency. These patients may also present with arrhythmias secondary to an enlarged, hypertensive atrium.

Physical

Despite the intracardiac defect, no characteristic murmur or pathognomonic physical characteristics are present. This often leads to incorrect diagnosis of primary pulmonary vascular or parenchymal disease.

Signs of pulmonary venous obstruction and pulmonary hypertension are present. A right ventricular lift and accentuation of the pulmonary second sound are frequent and may be accompanied by an early diastolic murmur of pulmonary insufficiency. Rales may be present in the lung bases.

A soft, continuous murmur may be present due flow across the membrane. A murmur at the left sternal border is heard in patients with an atrial septal defect and a left-to-right shunt. A diastolic rumble of mitral stenosis at the apex is generally not heard with cor triatriatum.

Low cardiac output manifests as pallor, tachypnea, and diminished peripheral pulses.

Children are typically small, suffering from poor weight gain.

Patients presenting later in life may be dyspneic with a history of frequent pulmonary infections. They may have signs of right-sided heart failure, including distended peripheral veins and hepatomegaly. Signs and symptoms of pulmonary hypertension may be severe.

Causes

No known risk factors or associated genetic abnormalities have been reported.

Embryologically, the common pulmonary vein is normally absorbed and incorporated into the left atrium. Incomplete absorption results in a fibromuscular membrane that subdivides the left atrium into 2 chambers resulting in cor triatriatum.

Observation that a left superior vena cava is frequently associated with this lesion has led some to propose impingement of the left superior vena cava to the developing left atrium as a potential pathogenesis.

DDx


Diagnostic Considerations

Important considerations

Do not fail to diagnose cor triatriatum and other structural abnormalities in children with pulmonary hypertensive disease.

Special concerns

Patients should have an experienced congenital cardiac surgeon to treat this rare lesion. One of the few late complications is recurrence secondary to incomplete resection of the intra-atrial diaphragm.

Differential Diagnoses

Workup

Laboratory Studies

No specific laboratory studies are indicated in patients with suspected cor triatriatum.

Electrocardiography

ECG findings are nonspecific and may range from normal in asymptomatic older patients to mimicking those findings of a patient with the clinical picture of pulmonary hypertension.

In some patients, ECG may reveal the following:

  • Atrial arrhythmias [8]

  • Right-axis deviation

  • Right atrial enlargement depicted by enlarged P waves

  • Right ventricular hypertrophy

Chest Radiography

Chest radiographic findings are usually nonspecific but may include pulmonary congestion with diffuse haziness or Kerley B lines and the ground glass pattern of acute pulmonary edema in hilar areas.

Patients may have mild cardiac enlargement and prominence of the pulmonary arterial segment.

The dilated proximal chamber may produce the appearance of left atrial enlargement.

Presence of an atrial septal defect or of an associated partial anomalous pulmonary venous connection adds pulmonary overcirculation to the pulmonary venous obstruction. The radiograph may then reveal significant right ventricular enlargement.

Echocardiography

Echocardiography is often sufficient for diagnosis and is the diagnostic modality of choice. See the image below.

Mean Doppler gradient of 7-8 mm Hg across left atrMean Doppler gradient of 7-8 mm Hg across left atrial membrane indicating mildly elevated pulmonary venous pressures. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.

The membrane dividing the left atrium can be visualized using 2-dimensional echocardiography, as can the presence of an associated atrial septal defect. The origin of each of the pulmonary veins should be identified to exclude the presence of anomalous pulmonary venous return.

The distinction between cor triatriatum and a supramitral ring should be made by the location of the left-atrial appendage. Differentiating between cor triatriatum and total anomalous pulmonary venous drainage to the coronary sinus may be difficult.

Common cardiac anomalies can also be demonstrated

Transesophageal echocardiography (TEE) and intracardiac echocardiography offer precise image definition and spatial relationship of the membrane.

TEE is very useful in larger and older patients in whom transthoracic images are suboptimal especially in visualizing the left atrium. [9]

Angiography

This test is generally indicated to assess pulmonary venous return and pulmonary arterial pressures. Because approximately 10% of patients have partial anomalous venous return, angiography is helpful in defining the precise venous anatomy.

When performed, catheterization generally reveals pulmonary hypertension in a degree that varies directly with the severity of obstruction to pulmonary venous drainage. [8Demonstration of a pressure gradient between the left atrium and capillary wedge pressure is classic.

The proximal chamber is visualized during the venous phase, and a delay then occurs before the true left atrium and left ventricle are visualized. The proximal chamber then remains opacified and does not contract with the distal chamber

Cardiac CT Scanning and MRI

Both imaging modalities provide with very detailed anatomic images. [101112MRI has the advantage of not subjecting the patient to radiation. [1314]

Procedures

In classic cor triatriatum, cardiac catheterization reveals pulmonary hypertension without a left-to-right shunt. Pulmonary wedge pressures are high and left atrial pressures are within normal limits. Angiography via pulmonary artery injection shows an opacified pulmonary venous chamber draining into the left atrium.

Histologic Findings

Histology plays no part in the diagnosis; however, pulmonary hypertension results in well-defined structural changes.

There may be pulmonary edema and alveolar hemorrhage with lymphatic dilatation. Increased pulmonary arterial muscularity is present very early, with increased thickness of the arterial wall and extension of muscle into the arterioles. Necrotizing arteriolitis may be present.

TREATMENT AND MANAGEMENT


Medical Care

The goal of medical care in cor triatriatum is to reduce the symptoms caused by pulmonary venous congestion until definitive surgical therapy can be performed.

Patients presenting in extremis should be operated on immediately after resuscitation, without time spent for prolonged medical therapy.

On occasion, extracorporeal membrane oxygenation is initiated to stabilize a patient.

Consultations

Obtain consultations with a pediatric cardiologist and a pediatric cardiac surgeon.

Transfer

Admit patients with cor triatriatum who have undergone surgical treatment to a pediatric ICU experienced in dealing with congenital cardiac defects.

Provide postoperative treatment of heart failure and pulmonary hypertension until pulmonary vascular resistance normalizes.

Diet and activity

No specific dietary restrictions are recommended.

Physical activity should not be limited in patients with early and complete correction.

Patients with persistent pulmonary or cardiac dysfunction secondary to long-standing disease may have moderate restriction of exercise tolerance.

Surgical Care

Surgery is the treatment of choice. In relatively recent years, interventional catheterization techniques have evolved and been used successfully in some patients. [15]

Surgical correction

Open correction is currently preferred over closed (percutaneous) procedures.

The procedure is performed on cardiopulmonary bypass through an atrial incision with complete resection of the diaphragm.

Interventional cardiology

The role of percutaneous balloon dilation in managing this condition remains to be determined.

Postoperative care

Admit patients with cor triatriatum who have undergone surgical treatment to a pediatric ICU experienced in dealing with congenital cardiac defects.

Provide postoperative treatment of heart failure and pulmonary hypertension until pulmonary vascular resistance normalizes.

MEDICATION


Medication Summary

Medical management in cor triatriatum is targeted toward associated elevation in pulmonary vascular resistance and heart failure. It is continued in the postoperative period until the pulmonary resistance falls and right ventricular performance improves. Mainstays of treatment are inotropic agents and diuretics.

Inotropic agents

Class Summary

Cardiac glycosides (eg, digoxin) increases myocardial contractility in patients with heart failure. Adrenergic and dopaminergic agents (eg, dopamine) provide myocardial support in the perioperative period for patients with heart failure. The more restrictive the connection between proximal and distal chambers, the more likely inotropic support is required. Numerous agents are available in this category.

Digoxin (Lanoxin)

Exerts its inotropic action by increasing the amount of intracellular calcium available during excitation-contraction coupling. It is one of numerous inotropic agents that can be used in infants with congenital cardiac defects. Other agents, such as dopamine (described below), are more appropriate for acute management of heart failure in ICU setting.

Dopamine (Intropin)

Adrenergic agonists are often used in the critical care setting for their rapid onset of action and rapid peak effect. They are, therefore, much easier to titrate to effect in acute settings. Their half-life is also much shorter than digoxin's, and their effects are rapidly lost when drug is discontinued.

Loop diuretics

Class Summary

These agents are used for management of right heart failure and pulmonary edema.

Furosemide (Lasix)

First-line drug for diuresis in newborns and infants and can be expected to be highly effective. It is a sulfonamide derivative that exerts its effects on the loop of Henle and distal renal tubule, thus inhibiting reabsorption of sodium and chloride.