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Unguarded tricuspid orifice and patent right ventricular outflow tract presenting with long-standing severe right heart failure in an adult
International Journal of Cardiology 66 (1998) 85–87
Unguarded tricuspid orifice and patent right ventricular outflow tract presenting with long-standing severe right heart failure in an adult Jagdish C. Mohan*, Rajiv Passey, R. Arora Department of Cardiology, G.B. Pant Hospital, New Delhi 110002, India Received 22 April 1998; accepted 28 May 1998
Abstract An adult female patient presenting with congestive heart failure was detected to have unguarded tricuspid valve orifice, patent right ventricular outflow tract and severe right ventricular systolic dysfunction on cross-sectional echocardiography. 1998 Elsevier Science Ireland Ltd. All rights reserved.
Unguarded tricuspid valve is a rare congenital anomaly with a very few antemortem case reports [1–6]. It is a variety of tricuspid valve dysplasia in which there is partial or complete agenesis of the tricuspid valvar tissue [2]. Most of the cases are associated with pulmonary atresia. The clinical presentation is usually in early childhood with cyanosis and / or congestive heart failure. This is a report of an unguarded tricuspid valve orifice and patent right ventricular outflow tract that presented as severe right heart failure in an adult female patient. To our knowledge, this case represents the oldest surviving patient with this anomaly and the second patient who presented with congestive heart failure. A 41-year-old Indian woman was admitted to the hospital with an 8 year history of exertional dyspnoea, fatigue and abdominal swelling. The symptoms began 2 years after the last childbirth which was uncomplicated. Pedal oedema ensued several months after abdominal swelling. Her symptoms had worsened over the last 6 months. Physical examination *Corresponding author. [email protected]
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191
11
6840256;
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revealed a dark-skinned oriental woman of medium height and weight with suffused face. Supine blood pressure was 100 / 70 mmHg, her pulse (88 / min) was irregular, jugular and facial veins were markedly distended, tortuous, cord-like and pulsatile. There was bilateral pedal oedema, mild clubbing of fingers, tense ascites and markedly pulsatile hepatomegaly. Precordial examination showed diffuse cardiac apex in 6th left intercostal space on anterior axillary line, systolic pulsations along upper left sternal border, normal first heart sound, wide fixed split second heart sound with diminished intensity of pulmonary component, a loud third heart sound at lower left sternal border and a grade 3 / 6 systolic murmur which was audible over a wide area. There was a faint middiastolic murmur at the lower sternal edge. The 12lead electrocardiogram showed mean frontal QRS axis of 1105*, atrial fibrillation and incomplete right bundle branch block. Plain chest skiagram showed massive, globular shape of cardiac silhouette and pulmonary oligemia (Fig. 1). Routine plasma biochemical parameters were normal. 2D-echocardiography (Fig. 2) revealed markedly dilated right atrium, right ventricle, right ventricular outflow tract, inferior
0167-5273 / 98 / $19.00 1998 Elsevier Science Ireland Ltd. All rights reserved. PII: S0167-5273( 98 )00150-8
86
J.C. Mohan et al. / International Journal of Cardiology 66 (1998) 85 – 87
Fig. 1. Plain chest skiagram in anteroposterior view showing massive globular cardiomegaly and mild pulmonary oligemia.
Fig. 2. Composite 2D-echocardiographic study showing dilated right atrium, right ventricle and the patent right ventricular outflow tract. Tricuspid orifice is unguarded with virtually no septal leaflet. Lower right panel shows systolo-diastolic flow across the tricuspid orifice with low velocities.
vena cava with absent respiratory variation, normal left atrium, pulmonary artery and the left ventricle. Tricuspid annular diameter was 4.8 cm, mural tricuspid leaflet was absent and the anterior tricuspid leaflet was 6 mm in length. Interatrial septum bowed to the left. Global right ventricular systolic function was markedly reduced with normal left ventricular ejection fraction (62%). Systemic and pulmonary venous drained normally and the pulmonary valve was structurally normal.Colour Doppler examination revealed severe tricuspid regurgitation with laminar flow, mild pulmonary regurgitation and trace mitral regurgitation. Continuous-wave Doppler interrogation of the right ventricular inflow showed systolo-diastolic flow with antegrade peak velocity of ,1.0 m / s and retrograde systolic peak velocity of 1.5 m / s. End-diastolic velocity of the pulmonary regurgitation signal averaged 0.5 m / s. Multiplane transesophageal examination did not contribute any additional information. The patient declined further invasive procedures and surgery. Congenitally unguarded tricuspid orifice is a rare anomaly with about 10 cases reported in the literature with seven antemortem diagnosis [1–6]. Isolated unguarded tricuspid orifice with no other congenital abnormality has been reported in three patients so far [1,3,4]. All except one patient presented with cyanosis [4]. The patient reported by Magotra et al. [4], like the patient in this case report, presented with intractable right heart failure. No patient surviving till adulthood has been reported. Prolonged history of right heart failure, atrial fibrillation and onset of symptoms in adult life are unique features of the present case. Partial or complete absence of tricuspid valvar tissue diagnosed on fetal echocardiography was labelled as unguarded tricuspid valve orifice by Kanjuh and coworkers in 1964 [7]. The condition needs to be differentiated from Ebstein’s malformation, tricuspid dysplasia in association with pulmonary atresia and intact ventricular septum and Uhl’s anomaly. Dysplasia of the leaflets along with displacement of the septal leaflet is an integral part of Ebstein malformation. However the mural leaflet is always present unlike in unguarded tricuspid orifice wherein it may be completely absent. Pulmonary atresia with intact interventricular septum may be associated with vari-
J.C. Mohan et al. / International Journal of Cardiology 66 (1998) 85 – 87
able degree of tricuspid valve dysplasia but needs to be differentiated from the Ebstein malformation or the unguarded tricuspid orifice only if the right ventricle is dilated The tricuspid valve is structurally normal in Uhl’s anomaly. Dysplasia of the tricuspid valve is probably the most common cause of isolated tricuspid regurgitation and the unguarded tricuspid orifice is its extreme form. Because of the poor right ventricular contractile function, the pulmonary circulation is maintained by the pumping action of the right atrium or the outflow tract. The right atrium can assume enormous proportions as in the present case and right-to-left shunting can occur through patent foramen ovale. In some of these cases, functional pulmonary atresia can occur, resulting from a combination of a severely abnormal tricuspid valve and markedly depressed right ventricular contractility.
References [1] Mohan JC, Tatke M, Arora R. Rudimentary dysplastic valvar tissue guarding the tricuspid orifice with dilatation of the right ventricle and a patent outflow tract. Int J Cardiol 1989;25:136–9.
87
[2] Anderson RH, Silverman NH, Zuberbuhler JR. Congenitally unguarded tricuspid orifice: its differentiation from Ebstein’s anomaly in association with pulmonary atresia and intact ventricular septum. Pediatr Cardiol 1990;11:86–90. [3] Ozkutlu S, Gunal S, Caglar M, Alehan D, Gungor C. Unguarded tricuspid orifice: a rare malformation of tricuspid valve diagnosed by echocardiography. Report of two cases and review of the literature. Int J Cardiol 1996;56:125–9. [4] Magotra RA, Agrawal NB, Mall SP, Parikh SJ. Severe dysplasia of the tricuspid valve (unguarded tricuspid annulus): clinical presentation and surgical treatment. J Thorac Cardiovasc Surg 1990;99:174– 5. [5] Munoz-Castellanos L, Salinas CH, Kuri-Nivon M, Garcia-Arenal F. Absence of the tricuspid valve. A case report. Arch Inst Cardiol Mex 1992;62:61–7. [6] Gussenhoven EJ, Essed CE, Bos E. Unguarded tricuspid orifice with two-chambered right ventricle. Pediatr Cardiol 1986;7:175–7. [7] Kanjuh VI, Stevenson JE, Amplatz K, Edwards JE. Congenitally unguarded tricuspid valve orifice with coexistent pulmonary atresia. Circulation 1964;30:911–7.
Unguarded tricuspid orifice and patent right ventricular outflow tract presenting with long-standing severe right heart failure in an adult Jagdish C. Mohan*, Rajiv Passey, R. Arora Department of Cardiology, G.B. Pant Hospital, New Delhi 110002, India Received 22 April 1998; accepted 28 May 1998
Abstract An adult female patient presenting with congestive heart failure was detected to have unguarded tricuspid valve orifice, patent right ventricular outflow tract and severe right ventricular systolic dysfunction on cross-sectional echocardiography. 1998 Elsevier Science Ireland Ltd. All rights reserved.
Unguarded tricuspid valve is a rare congenital anomaly with a very few antemortem case reports [1–6]. It is a variety of tricuspid valve dysplasia in which there is partial or complete agenesis of the tricuspid valvar tissue [2]. Most of the cases are associated with pulmonary atresia. The clinical presentation is usually in early childhood with cyanosis and / or congestive heart failure. This is a report of an unguarded tricuspid valve orifice and patent right ventricular outflow tract that presented as severe right heart failure in an adult female patient. To our knowledge, this case represents the oldest surviving patient with this anomaly and the second patient who presented with congestive heart failure. A 41-year-old Indian woman was admitted to the hospital with an 8 year history of exertional dyspnoea, fatigue and abdominal swelling. The symptoms began 2 years after the last childbirth which was uncomplicated. Pedal oedema ensued several months after abdominal swelling. Her symptoms had worsened over the last 6 months. Physical examination *Corresponding author. [email protected]
Fax:
191
11
6840256;
e-mail:
revealed a dark-skinned oriental woman of medium height and weight with suffused face. Supine blood pressure was 100 / 70 mmHg, her pulse (88 / min) was irregular, jugular and facial veins were markedly distended, tortuous, cord-like and pulsatile. There was bilateral pedal oedema, mild clubbing of fingers, tense ascites and markedly pulsatile hepatomegaly. Precordial examination showed diffuse cardiac apex in 6th left intercostal space on anterior axillary line, systolic pulsations along upper left sternal border, normal first heart sound, wide fixed split second heart sound with diminished intensity of pulmonary component, a loud third heart sound at lower left sternal border and a grade 3 / 6 systolic murmur which was audible over a wide area. There was a faint middiastolic murmur at the lower sternal edge. The 12lead electrocardiogram showed mean frontal QRS axis of 1105*, atrial fibrillation and incomplete right bundle branch block. Plain chest skiagram showed massive, globular shape of cardiac silhouette and pulmonary oligemia (Fig. 1). Routine plasma biochemical parameters were normal. 2D-echocardiography (Fig. 2) revealed markedly dilated right atrium, right ventricle, right ventricular outflow tract, inferior
0167-5273 / 98 / $19.00 1998 Elsevier Science Ireland Ltd. All rights reserved. PII: S0167-5273( 98 )00150-8
86
J.C. Mohan et al. / International Journal of Cardiology 66 (1998) 85 – 87
Fig. 1. Plain chest skiagram in anteroposterior view showing massive globular cardiomegaly and mild pulmonary oligemia.
Fig. 2. Composite 2D-echocardiographic study showing dilated right atrium, right ventricle and the patent right ventricular outflow tract. Tricuspid orifice is unguarded with virtually no septal leaflet. Lower right panel shows systolo-diastolic flow across the tricuspid orifice with low velocities.
vena cava with absent respiratory variation, normal left atrium, pulmonary artery and the left ventricle. Tricuspid annular diameter was 4.8 cm, mural tricuspid leaflet was absent and the anterior tricuspid leaflet was 6 mm in length. Interatrial septum bowed to the left. Global right ventricular systolic function was markedly reduced with normal left ventricular ejection fraction (62%). Systemic and pulmonary venous drained normally and the pulmonary valve was structurally normal.Colour Doppler examination revealed severe tricuspid regurgitation with laminar flow, mild pulmonary regurgitation and trace mitral regurgitation. Continuous-wave Doppler interrogation of the right ventricular inflow showed systolo-diastolic flow with antegrade peak velocity of ,1.0 m / s and retrograde systolic peak velocity of 1.5 m / s. End-diastolic velocity of the pulmonary regurgitation signal averaged 0.5 m / s. Multiplane transesophageal examination did not contribute any additional information. The patient declined further invasive procedures and surgery. Congenitally unguarded tricuspid orifice is a rare anomaly with about 10 cases reported in the literature with seven antemortem diagnosis [1–6]. Isolated unguarded tricuspid orifice with no other congenital abnormality has been reported in three patients so far [1,3,4]. All except one patient presented with cyanosis [4]. The patient reported by Magotra et al. [4], like the patient in this case report, presented with intractable right heart failure. No patient surviving till adulthood has been reported. Prolonged history of right heart failure, atrial fibrillation and onset of symptoms in adult life are unique features of the present case. Partial or complete absence of tricuspid valvar tissue diagnosed on fetal echocardiography was labelled as unguarded tricuspid valve orifice by Kanjuh and coworkers in 1964 [7]. The condition needs to be differentiated from Ebstein’s malformation, tricuspid dysplasia in association with pulmonary atresia and intact ventricular septum and Uhl’s anomaly. Dysplasia of the leaflets along with displacement of the septal leaflet is an integral part of Ebstein malformation. However the mural leaflet is always present unlike in unguarded tricuspid orifice wherein it may be completely absent. Pulmonary atresia with intact interventricular septum may be associated with vari-
J.C. Mohan et al. / International Journal of Cardiology 66 (1998) 85 – 87
able degree of tricuspid valve dysplasia but needs to be differentiated from the Ebstein malformation or the unguarded tricuspid orifice only if the right ventricle is dilated The tricuspid valve is structurally normal in Uhl’s anomaly. Dysplasia of the tricuspid valve is probably the most common cause of isolated tricuspid regurgitation and the unguarded tricuspid orifice is its extreme form. Because of the poor right ventricular contractile function, the pulmonary circulation is maintained by the pumping action of the right atrium or the outflow tract. The right atrium can assume enormous proportions as in the present case and right-to-left shunting can occur through patent foramen ovale. In some of these cases, functional pulmonary atresia can occur, resulting from a combination of a severely abnormal tricuspid valve and markedly depressed right ventricular contractility.
References [1] Mohan JC, Tatke M, Arora R. Rudimentary dysplastic valvar tissue guarding the tricuspid orifice with dilatation of the right ventricle and a patent outflow tract. Int J Cardiol 1989;25:136–9.
87
[2] Anderson RH, Silverman NH, Zuberbuhler JR. Congenitally unguarded tricuspid orifice: its differentiation from Ebstein’s anomaly in association with pulmonary atresia and intact ventricular septum. Pediatr Cardiol 1990;11:86–90. [3] Ozkutlu S, Gunal S, Caglar M, Alehan D, Gungor C. Unguarded tricuspid orifice: a rare malformation of tricuspid valve diagnosed by echocardiography. Report of two cases and review of the literature. Int J Cardiol 1996;56:125–9. [4] Magotra RA, Agrawal NB, Mall SP, Parikh SJ. Severe dysplasia of the tricuspid valve (unguarded tricuspid annulus): clinical presentation and surgical treatment. J Thorac Cardiovasc Surg 1990;99:174– 5. [5] Munoz-Castellanos L, Salinas CH, Kuri-Nivon M, Garcia-Arenal F. Absence of the tricuspid valve. A case report. Arch Inst Cardiol Mex 1992;62:61–7. [6] Gussenhoven EJ, Essed CE, Bos E. Unguarded tricuspid orifice with two-chambered right ventricle. Pediatr Cardiol 1986;7:175–7. [7] Kanjuh VI, Stevenson JE, Amplatz K, Edwards JE. Congenitally unguarded tricuspid valve orifice with coexistent pulmonary atresia. Circulation 1964;30:911–7.