Korean Circ J.  2015 Jul;45(4):340-343. 10.4070/kcj.2015.45.4.340.

Ruptured Tricuspid Valve Papillary Muscle in a Neonate with Intractable Persistent Fetal Circulation

• Yoon JK
• Kim HR
• Kwon HW
• Kwon BS
• Kim GB
• Bae EJ
• Noh CI
• Kim WH

• KMID: 1964108
• DOI: http://doi.org/10.4070/kcj.2015.45.4.340

Abstract

Unguarded tricuspid regurgitation (TR) due to a flail tricuspid leaflet is a rare condition of newborn cyanosis. A high perinatal mortality has been associated with this fatal condition. But, there are feasible surgical repairs to improve survival. We report the case of a male full-term neonate with intractable hypoxia. He had profound tricuspid insufficiency and leaflet prolapse caused by a ruptured papillary muscle supporting the anterior leaflet of the tricuspid valve. He presented with severe cyanosis and respiratory distress immediately after birth. Despite medical management, the pulmonary vascular resistance was not decreased and a low cardiac output persisted. Initial stabilization was accomplished with nitric oxide and extracorporeal membrane oxygenation. The tricuspid valve repair surgery was successfully performed subsequently. TR resulting from papillary muscle rupture is a potentially lethal condition. Timely diagnosis and proper surgical treatment can be lifesaving.

Keyword

Cyanosis; Neonate; Tricuspid regurgitation; Papillary muscles; Cardiac surgery

MeSH Terms

Anoxia
Cardiac Output, Low
Cyanosis
Diagnosis
Extracorporeal Membrane Oxygenation
Female
Humans
Infant, Newborn*
Male
Nitric Oxide
Papillary Muscles*
Parturition
Perinatal Mortality
Persistent Fetal Circulation Syndrome*
Prolapse
Rupture
Thoracic Surgery
Tricuspid Valve Insufficiency
Tricuspid Valve*
Vascular Resistance
Nitric Oxide

Figure

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  Fig. 1 Anterior-posterior chest radiograph. A: on admission, a right pneumothorax with mediastinal shifting is observed. B: after chest tube insertion for the pneumothorax, cardiomegaly is not severe. C: thirty hours after birth, cardiomegaly has not progressed; however, the pulmonary vascular marking has decreased.

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  Fig. 2 Apical four chamber echocardiographic image shows a flail anterior leaflet of the tricuspid valve. The thick end (arrow, A) of the leaflet is the ruptured papillary muscle, which is documented more clearly with cine imaging (B), and the severe tricuspid regurgitation is documented by color Doppler imaging (C). LA: left atrium, LV: left ventricle, RA: right atrium, RV: right ventricle, TV: tricuspid valve.

• 

  Fig. 3 The neonate shows intractable hypoxemia and cardiac shock despite treatment. After ECMO support and surgery, the vital signs stabilize, and the demand of inotropic drugs, NO, and oxygen is decreased. SpO2: saturation by pulse oximetry, HR: heart rate, SBP: systolic blood pressure, CPR: cardiopulmonary resuscitation, HFOV: high frequency oscillatory ventilator, ECMO: extracorporeal membrane oxygenation.

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