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Positional cyanosis in infants: an unusual presentation of right-sided cardiac masses
Published online by Cambridge University Press: 20 January 2005
Abstract
Positional cyanosis is an uncommon finding in young patients. We report three infants who presented with positional cyanosis due to a pedunculated tumour in the right heart. Arterial desaturation was the result of right-to-left shunting at the level of the oval foramen caused by obstruction and/or insufficiency of the tricuspid valve. The obstruction at the level of the tricuspid valve was variable because of the pedunculated nature of the tumours, which gave them considerable mobility. Hence, the degree of right-to-left shunting was dependent on the position of the patient. In all the patients, surgical resection of the tumours resolved the cyanosis.
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- © 2004 Cambridge University Press
Cyanosis in infancy is a major physical sign. Its cause needs to be diagnosed rapidly if deterioration and complications are to be prevented. Positional cyanosis is one variant of this feature that appears only with certain orientations of the body. We report three infants with right-sided intracardiac masses whose arterial saturation varied with changes in posture. Resection of the masses terminated the cyanotic episodes.
Case reports
Case 1
A girl was born at 3320 g by vaginal delivery, after 39 weeks of uncomplicated gestation. Apgar scores were 9 and 10 at 1 and 5 min, respectively. Twelve hours after birth, her mother noticed that her colour was blue while she was lying on her back, returning to pink when she was rolled onto her stomach. Physical examination in the supine position revealed a full-term baby with no respiratory distress. The heart rate was 143 beats/min, and the respiratory rate was 48 breaths/min. Oxygen saturation varied between 65 and 70%, and did not improve with 100% oxygen. The peripheral pulses and heart sounds were normal, with no audible murmur. Turning the baby onto her stomach raised her oximetric saturation to 96%. Chest radiography showed mild cardiomegaly with normal pulmonary vascularity. Echocardiography while the baby was in a right lateral decubitus position showed a large pedunculated hyperechogenic mass protruding from the right atrium across the tricuspid valve into the right ventricle, with mild tricuspid valvar regurgitation. The mass originated from the lower aspect of the right atrial septum. There was left-to-right shunting across the oval foramen. Echocardiographic re-examination in the supine position revealed the flow across the tricuspid valve to be severely obstructed. There was Doppler evidence for antegrade flow across the tricuspid valve, and right-to-left shunting across the oval foramen. No other intracardiac masses were apparent. A large channel of communication between the pulmonary trunk and the aorta was also noted that permitted continuous left-to-right flow.
The baby underwent open-heart surgery, and the mass was completely removed. The tricuspid valve was structurally and functionally normal. Pathological examination showed the mass to be a myxoma.
Case 2
A 5-month-old baby boy, one of non-identical twins, was hospitalized after presenting with prolonged cyanotic episodes associated with severe respiratory distress. The weight at birth was 2300 g, the baby being born at 37 weeks of gestation. Growth and development were unremarkable. The mother recalled that, at 3 months of age, the baby preferred to lie on his stomach, and became agitated and dyspnoeic while in the supine position. The baby had been seen by a pediatrician, who suspected gastro-oesophageal reflux or breath-holding spells, and had been managed accordingly with no apparent improvement.
On admission, there was moderate cyanosis associated with respiratory distress when the baby was in the supine position. The respiratory rate was 66 breaths/min, the heart rate was 164 beats/min, and saturation of oxygen in room air was 67%. Peripheral pulses were normal. A Grade 1/6 systolic murmur was audible maximally over the left upper sternal border. The lungs were clear. When the baby was placed in the prone position, the symptoms immediately diminished and he calmed down. The saturation of oxygen increased to 94%, and respiratory distress was diminished, the heart rate dropping to 140 beats/min, and the respiratory rate to 48 breaths/min. A Grade 2/6 systolic ejection murmur was then audible. Bedside echocardiography with the baby in the supine position showed a pedunculated mass measuring 3 by 4 cm originating from the septal surface of the right ventricular cavity, and giving rise to severe obstruction of the subpulmonary outflow tract. Echo Doppler interrogation revealed a gradient of 92 mmHg, while colour Doppler examination revealed a right-to-left shunt at the atrial level, with severe tricuspid regurgitation. A repeated echocardiographic examination with the infant in the lateral or prone position showed relief of the outflow obstruction, and left-to-right shunting at atrial level. The mass was removed under cardiopulmonary by-pass, and was found to measure 4 by 3 by 1.5 cm. Pathologic examination again revealed a myxoma.
Case 3
A three-month-old baby boy was born at 37 weeks gestation with a birth weight of 3560 g. Ten days before admission he suffered from an upper respiratory infection with cough and fever. The antigen to the respiratory syncytial virus was positive. Because of clinical deterioration, he was admitted to the ward. Initial saturations of oxygen were 88% in room air. Physical examination revealed mild respiratory distress, with a heart rate of 163 beats/min, a respiratory rate of 52 breaths/min, and blood pressure of 85/44 mmHg. The heart sounds were normal, with no murmurs. Chest X-ray showed a normal cardiac silhouette, mild peribronchial thickening, and atelectasis of the right middle lobe. Positional cyanosis was noted in the supine position, with the saturation falling to 68%. In the prone position, the oxygen saturation immediately increased to 95%. Flexible bronchoscopy was performed, and showed mild softening of the tissues of the larynx.
Echocardiography showed a round mass measuring 3 by 3 cm that occupied nearly four-fifths of the cavity of the right atrium, originating from the lower aspect of the atrial free wall, and obstructing flow across the tricuspid valve. There was right-to-left shunting across the patent oval foramen (Fig. 1a). A repeated echocardiographic examination performed in the prone position showed only a mild degree of obstruction to flow across the tricuspid valve, with predominant left-to-right shunting across the oval foramen (Fig. 1b).
Figure 1. Sub-costal cross-sectional echocardiograms in our third patient showing changes in configuration of the cardiac mass. Whilst the patient was supine (a) there was right-to-left shunting through the patent oval foramen (arrow). When the patient was turned into the prone position (b) the shunt was left-to-right.
The mass was completely removed at surgery. Pathologic examination revealed an inflammatory mass consisting of cellular myofibroblastic proliferation.
Discussion
Primary and secondary intracardiac tumours are extremely rare at all ages, and occur mainly in infants and children. Primary tumours are more common, and include rhabdomyomas, fibromas and myxomas.1–3 Thrombuses and infected masses have been described in newborn babies, and in children with a hyperviscous haemodynamic state, or as part of a generalized infection.4, 5 Intracardiac masses may present as a cardiac murmur, or with arrhythmias, cardiac failure, or embolization. Cyanosis is a rarely reported manifestation of cardiac tumours. Conversely, cardiac tumours are among the least common causes of cyanosis in infancy, with only sporadic reports of isolated cases.6
Typically, such masses occupy the ventricular cavity, mimicking functionally univentricular physiology, or obstructing the right ventricular outflow tract and simulating pulmonary atresia. Alternatively, tricuspid valvar function may be disturbed by the mass, causing insufficiency and/or obstruction. In this setting, the pathophysiology of cyanosis is similar to that seen in Ebstein's malformation, in which severe tricuspid insufficiency increases the right atrial pressure, thus causing right-to-left atrial shunting. Cross-sectional echocardiography is a valuable tool for rapid recognition and diagnosis of intracardiac masses, and for identifying associated tricuspid valvar dysfunction as the cause of cyanosis.
Our 3 patients were particularly unusual, in that the cyanosis was variable and dependent on body position. Doppler echocardiography showed varying degrees of tricuspid obstruction and insufficiency, and consequently variable right-to-left shunting with different bodily positions. Two of the masses we identified were found to be myxomas, and one was an infected thrombus. Although the rhabdomyoma is the most common cardiac tumour in childhood, atrial and ventricular myxomas have also been reported in infancy.7 Of such tumours found in infants, three-quarters originate from the left atrium, with most of the remainder occurring in the right atrium, and only rarely in the right ventricle. The tumour can have a broad base of attachment or be pedunculated. Data collected from 25 adults showed congestive heart failure to be the presenting symptom in 16, palpitations in eight, neurologic symptoms in six, and constitutional symptoms in three.8 Severe cyanosis has also been described in a neonate.9
Infected intracardiac masses in children have historically been considered a complication of congenital or rheumatic disease. The use of prophylactic anti-biotics, and the decline in rheumatic fever, have now made infected masses a less common finding, being confined primarily to hospitalized patients, especially those who are managed with indwelling central venous catheters.10, 11 The inflammatory pseudo tumour, also known as the inflammatory myofibroblastic tumour, is an uncommon spindle-cell lesion that was initially recognized in the lung, and is now known to occur in virtually every major organ of the body. Its precise biological nature is still uncertain.12 The inflammatory mass in our patient was presumably related to his antecedent febrile illness, but its source and etiology remain unproven.
Surgical resection is indicated as soon as a diagnosis is established in all symptomatic patients with positional cyanosis due to intracardiac masses. Removal of the tumour relieves the obstruction, be it at the level of the ventricular inflow or outflow, and resolves the cyanosis. This was shown in both our patients with atrial myxomas. In our third patient, the obstructing mass was found to be an infected thrombus. In this instance, combined antithrombotic and antibiotic treatment could have been an alternative mode of treatment, as described previously.13 It proved impossible, nonetheless, to differentiate echocardiographically the thrombus from a tumour, and surgical resection is always a logical approach in a symptomatic baby, as had proved the case in our previous patients.
Positional cyanosis is a rare finding in infants, and our experience indicates that intracardiac masses should be included in its differential diagnosis. Sudden cyanosis, or haemodynamic collapse induced by changes in position during nursing and transport, can readily be prevented once the diagnosis is established. Echocardiography is the most valuable diagnostic tool, and should be used promptly and liberally in any similar or suspicious cases.
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Sub-costal cross-sectional echocardiograms in our third patient showing changes in configuration of the cardiac mass. Whilst the patient was supine (a) there was right-to-left shunting through the patent oval foramen (arrow). When the patient was turned into the prone position (b) the shunt was left-to-right.
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