Cor triatriatum in adults

Cor triatriatum in adults

472 4. 5. 6. 7. 8. 9. 10. Horowitz et al. with echocardiographically detected right-sided heart thrombi: a metaanalysis. AM HEARTJ 1989;118:569-...

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with echocardiographically detected right-sided heart thrombi: a metaanalysis. AM HEARTJ 1989;118:569-73. Kronik G, for European Working Group on Echocardiography. The European cooperative study on the clinical significance of right heart thrombi. Eur Heart J 1989;10:1046-59. Kasper W, Meinertz T, Henkel B, Eissner D, Hahn K, Hofmann T, Zeiher A, Just H. Echocardiographic findings in patients with proved pulmonary embolism. AM HEART J 1986;112:1284-90. Franzoni P, Cuccia C, Zappa C, Volpini M, Gel P, Visioli O. Tromboembolo migrante nelle cavit~ cardiache destre in corso di embolia polmonare. G Ital Cardiol 1989;19:7-16. Chakko S, Richards F. Right-sided cardiac thrombi and pulmonary embolism. Am J Cardiol 1987;59:195. Goldhaber SZ, Heit J, Sharma GVRK, Friedenberg WR, Heiselman DE, Wilson DB, Parker JA, Bennet D, Feldstein ML, Selwyn AP, Kim D, Sharma GVRK, Nagel JS, Meyerovitz MF. Randomized controlled trial of recombinant tissue plasminogen activator versus urokinase in the treatment of acute pulmonary embolism. Lancet 1988;2:293-8. Goldhaber SZ, Kessler CM, Heir JA, Elliot CG, et al. Recombinant tissue-type plasminogen activator versus a novel dosing regimen of urokinase in acute pulmonary embolism: a randomized controlled multicenter trial. J Am Coll Cardiol 1992;20:24-30. Levine MN, Hirsh J, Weitz J, Cruikshank M, Neemeh J, Turpie AG, Gent M. A randomized trial of a single bolus dosage regimen of recombinant tissue plasminogen activator in patients with acute pulmonary embolism. Chest 1990;98: 1473-9.

Cor triatriatum in adults Michael D. Horowitz, MD, W a r r e n Zager, BA, M a r t i n Bilsker, MD, a Richard A. Perryman, MD, and Maureen H. Lowery, MD a Miami, Fla.

Cot t r i a t r i a t u m , first described in 1868 by Church, 1 is an uncommon congenital cardiac anomaly. 2, 3 In cor triatriaturn, a fibromuscular membrane subdivides the left atrium into a posterosuperior chamber t h a t receives blood from the pulmonary veins and an anteroinferior chamber (the true left atrium) t h a t contains the left atrial appendage and the mitral valve orifice.2, 4, 5 In most cases, the two chainbers communicate through one or more small perforations in the intraatrial membrane. Although cot t r i a t r i a t u m is most commonly seen in children, it is known to occur in adults.2-4, 6-9 In this report, we present a case of cot triatriatum in a 34-year-old man. T h e p a t i e n t underwent excision of the intraatrial membrane, with an excellent outcome. A 34-year-old man was referred to Jackson Memorial. Hospital for evaluation and t r e a t m e n t of suspected pulmonary hypertension. The p a t i e n t was in a p p a r e n t good health until age 29, at which time he began to have episodes of coughing associated with hemoptysis. Although these

From the Division of Thoracic and Cardiovascular Surgery and athe Division of Cardiology, University of Miami/Jackson Memorial Medical Center. Reprint requests: Michael D. Horowitz, MD, 4211 Hospital Road, Suite 302, Fascagoula, MS 39581. AM HEART J 1993;126:472-474 Copyright © 1993 by Moshy -Year Book, Inc. 0002-8703/93/$1.00 + .10 4/4/47081

August 1993 American Heart Journal

symptoms occurred sporadically over a period of 5 years, the patient did not seek medical attention. A p p r o x i m a t e l y 6 months before admission he had an episode of pneumonia for which he was treated with oral antibiotics as an outpatient. The p a t i e n t was hospitalized elsewhere for an a p p a r e n t seizure. He was transferred to this institution when he was incidentally found to have signs and symptoms of pulmonary hypertension. The p a t i e n t stated t h a t he had night sweats and t h a t he had lost 25 pounds over the preceding months. He a d m i t t e d to smoking two to three packs of cigarettes per day. His past history was otherwise unremarkable. Physical examination showed a thin man in no acute distress. T h e h e a r t rate was 84 per minute, the blood pressure was 130/90 m m Hg, and his respiratory rate was 18 per minute. Jugular venous pulsations had a prominent v wave. The arterial pulses were full. A strong right ventricular heave was palpated. The first heart sound was normal. The pulmonic component of the second heart sound was accentuated. There was a III/VI holosystolic m u r m u r best heard at the left lower sternal border t h a t became louder with inspiration. There was a II/VI diastolic m u r m u r heard at the left upper sternal border t h a t also increased with inspiration. Auscultation of the chest revealed scattered tales bilaterally. There was no a d e n o p a t h y or hepatosplenomegaly. The remainder of the examination was normal. The admission laboratory studies were normal except for a hematocrit of 39 % and an erythrocyte sedimentation rate of 72 ram/hr. Arterial blood gas showed a mild respiratory alkalosis. The room air Pao2 was 78 torr. A chest x-ray film showed bilateral increased interstitial markings and hilar fullness. The electrocardiogram showed normal sinus rhythm, left atrial enlargement, right axis deviation, and right ventricular h y p e r t r o p h y with a strain pattern. A ventilation-perfusion lung scan showed decreased perfusion bilaterally, but there were no focal abnormalities to suggest pulmonary embolism. A computed tomography (CT) scan showed bilateral hilar adenopathy; no abnorrealities were visualized in the abdomen and pelvis. Transthoracic and transesophageal echocardiograms demonstrated a membrane subdividing the left atrium superior to the atrial appendage (Fig. 1). There was evidence of severe pulmonary hypertension. The left and right ventricular function was normal. Pressures obtained at the time of cardiac catheterization are shown in Table I. The pulmonary vascular resistance was markedly elevated at 571 dynes/sec/cm -5. The cardiac index was 2.4 L / m i n / m 2. Surgical correction of cor t r i a t r i a t u m was performed. The intraatrial membrane was excised except for a calcified rim adjacent to the left atrial wall. The mitral valve was normal to inspection. Histopathologic examination of enlarged mediastinal l y m p h nodes showed reactive hyperplasia with no neoplasm or granuloma. The postoperative recovery was uncomplicated. Physical examination subsequent to surgery revealed no auscultatory evidence of tricuspid or pulmonic valve insufficiency. An echocardiogram performed several days postoperatively showed normal biventricular function. T h e estimated right ventricular systolic pressure had decreased to 35 m m Hg. The p a t i e n t was discharged home 6 days after surgery. He was doing well 25 months postoperatively.

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Fig. 1. Preoperative transesophageal echocardiogram in four-chamber view shows membrane (MEM) dividing the left atrium. AO, Aorta; LA, left atrium; LV, left ventricle; MV, mitral valve; R V, right ventricle.

Cor t r i a t r i a t u m is a rare congenital cardiac malformation. It is diagnosed in approximately 0.1% of children who have congenital heart disease. 5 Approximately 70 % to 80 % of children with cor t r i a t r i a t u m have other cardiac defectsfl 7 Cor t r i a t r i a t u m is occasionally encountered in adults.2-4, 6-9 In fact, this congenital anomaly has been diagnosed in patients over t h e age of 50 years. 2, 6-8 In cor triatriatum, a fibromuscular membrane subdivides the left atrium into two chambers. The posterosuperior chamber receives blood from the pulmonary veins. The anteroinferiot chamber, the true left atrium, contains the left atrial appendage and mitral valve orifice.4 Communication between the two chambers usually occurs through one or more perforations in the membrane. Cot t r i a t r i a t u m occurs in several forms. Loeffler 2 classified hearts with cor triatriat u m into three groups depending on the size of the opening(s) in the intraatrial membrane. T y p e 1 contained no opening in the membrane; type 2 had a small opening in the membrane; type 3 had a large communication through the membrane. In patients with type 1 or 2 cor triatriatum, there is obstruction to pulmonary venous return. However, when there is a large communication across the membrane, hemodynamics may be normal and the patient may be asy m p t o m a t i c in childhood. Defec£s in the interatrial septum are common in patients with cor triatriatum. 7 When the septal defect exists between the right atrium and the posterosuperior (inflow) chamber, blood is shunted from left to right. W h e n there is communication between the right atrium and the anteroinferior (outflow) chamber, a rightto-left shunt occurs. 3, 5 In some cases there is communication between all three chambers through septal defects above and below the membrane. 7 Symptoms and signs of isolated cor t r i a t r i a t u m are similar to those of mitral valve stenosis. 5 In patients with additional cardiac defects, the presentation may be quite

T a b l e I. Intracavitary pressures in a 34-year-old man with isolated cor t r i a t r i a t u m

Site Right atrium Right ventricle Pulmonary artery Pulmonary capillary wedge Left ventricle Aorta

Pressure (ramHg) (5) 100/12 100/5 (65) (30) 100/5 100/60

Numbers in parentheses indicate mean pressures.

variable and is determined by the specific combination of lesions. Various techniques are available to identify the intraatrial membrane in patients with cor triatriatum. Transthoracic and transesophageal echocardiography are most commonly used because of their availability, safety, and accuracy. 4 Additional techniques for visualizing the intraatrial membrane include angiography, CT scanning, and magnetic resonance imaging. 6, 7,10 Cot t r i a t r i a t u m was incidentally diagnosed by a CT scan performed for evaluation of lung cancer in a 75-year-old man. 6 The t r e a t m e n t of cor t r i a t r i a t u m is surgical resection of the intraatrial membrane. In patients with additional cardiac defects, appropriate corrective or palliative procedures are performed during the same operation. The outcome for the patient with isolated cor t r i a t r i a t u m is excellentfl 5 REFERENCES

1. Church WS. Congenital malformation of the heart: abnormal septum in the left auricle. Trans Pathol Soc Lond 1868;19:18890. 2. Loeffier E. Unusual malformation of the left atrium: pulmonary sinus. Arch Pathol Lab Med 1949;48:371-6.

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3. Oglietti J, Cooley DA, Izquierdo JP, Ventemiglia R, Muasher I, Hallman GL, Reul Jr G. Cor triatriatum: operative results in 25 patients. Ann Thorac Surg 1983;35:415-20. 4. Schluter M, Burkhart LA, Wolfgang T, Langenstein BA, Thier W, Schmiegel W-H, Krebber H-J, Kalmar P, Hanrath P. Transesophageal two-dimensional echocardiography in the diagnosis of cor triatriatum in the adult. J Am Coll Cardiol 1983;2:1011-5. 5. Rodefeld MD, Brown JW, Heimansohn DA, King H, Girod DA, Hurwitz RA, Caldwell RL. Cor triatriatum: clinical presentation and surgical results in 12 patients. Ann Thorac Surg 1990;50:562-8. 6. Tanaka F, Itoh M, Esaki H, Isobe J, Inoue R. Asymptomatic cor triatriatum incidentally revealed by computed tomography. Chest 1991;100:272-4. 7. Thilenius OG, Bharati S, Lev M. Subdivided left atrium: an expanded concept of cor triatriatum sinistrum. Am J Cardiol 1976;37:743-52. 8. Macmillan RM, Rees MR, Maranhao V, Clark DL. Cine-computed tomography of cot triatriatum. J Comput Assist Tomogr 1986;10:124-5. 9. Cooley DA, Murphy MC. Cot triatriatum and anomalous pulmonary venous return. Tex Heart Inst J 1990;17:118-21. 10. Bisset GS, Kirks DR, Strife JL, Schwartz DC. Cor triatriatum: diagnosis by MR imaging. AJR 1987;149:567-8.

Transseptal balloon mitral valvuloplasty in mitral stenosis with atrial septal aneurysm Kuo-Ho Yeh, MD, Morgan Fu, MD, Chiung-Jen Wu, MD, Sarah O. Chua, MD, Yu-Ching Chen, MD, and Jui-Sung Hung, MD Taipei, Taiwan, Republic of China

Atrial septal aneurysm (ASA) is rare; it may occur as an anomaly or in association with other cardiac lesions such as mitral valve prolapse or ostium secundum atrial septal defect. 1-t In the English literature there have been only three reported cases of coexistent ASA and mitral stenosisJ, 5 One of the two patients reported by Hanley et al.4 was managed by surgical mitral commissurotomy. The patient of Tizon et al. 5 underwent mitral replacement and a Teflon patch repair of the interatrial septum after resection of the ASA. Percutaneous transvenous mitral commissurotomy ( P T M C ) - - m i t r a l valvuioplasty by means of Inoue balloon catheter--is a safe and effective treatment for severe mitral stenosis. 6 When mitral stenosis is associated with ASA, ASA poses a therapeutic challenge because transseptal catheterization is a prerequisite to PTMC. We report uncomplicated transseptal catheterization and P T M C in a patient with severe rheumatic mitral stenosis and ASA. A 36-year-old woman with severe rheumatic mitral stenosis and atrial fibrillation was admitted in May 1990 because of severe pulmonary venous congestion despite From the Section of Cardiology, Chang Gung Medical College and Chang Gung Memorial Hospital (Kaohsiung Medical Center), Taiwan. Reprint requests: Jui-Sung Hung, MD, Section of Cardiology, Chang Gung Memorial Hospital, 199 Tung Hwa North Road, Taipei 105, Taiwan, Republic of China. AM HEARTJ 1993;126:474-475 Copyright © 1993 by Mosby-Year Book, Inc. 0002-8703/93/$1.00 + .10 4 / 4 / 4 7 0 8 3

Fig. 1. Two-dimensional echocardiograms in parasternal short-axis view (top panel) and apical four-chamber view (bottom panel) show presence of atrial septal aneurysm (asa) with bulging of atrial septum into right atrium (RA) at level of fossa ovalis. Stenotic mitral valve (my) is also seen in apical four-chamber view (bottom panel). AO, Aorta; LA, left atrium; L V, left ventricle; R V, right ventricle.

treatment with digoxin and diuretics. Two-dimensional echocardiography showed severe stenosis of the pliable, noncalcified mitral valve and the presence of an ASA (Fig. 1). Color Doppler study showed no intracardiac shunts or mitral regurgitation. After giving informed consent, the patient underwent cardiac catheterization, which confirmed the presence of severe mitral stenosis. Frontal and lateral biplane right atrial angiograms elucidated the anatomic relationship between the atria and ASA, thus providing landmark guidance for transseptal catheterization, The puncture site was cephalid and dorsal to the ASA as was later also confirmed by left atrial angiograms (Fig. 2). P T M C was performed by using the Inoue balloon catheter technique as described previously. 6 The patient tolerated the procedure well, and there were no complications except for development of 2+ angiographic mitral regurgitation. The mean left atrial pressure decreased from 26 to 12 mm Hg, and the mitral valve gradient from 18 to 5 mm Hg. The