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Fig. 4. Top left, Subcostal frontal view of a patient with sinus venosus defect of the inferior vena cava type (circle). This cut is sufficiently inferior that the ventricles are not displayed. Note that the defect is adjacent to the right lower pulmonary vein entrance site. This is far to the right of where a CSSD would be. L, Left atrium; R, right atrium; rlpu, right lower pulmonary vein; S, superior. TOP right, Subcostal left oblique view of the same case. The arrowheads show the sinus venosus defect of the inferior vena cava type. Bottom left, A subcostal left oblique view slightly further to the left shows the roof of the coronary sinus to be intact. White lines point to the thin roof. A, R, Anterior and rightward; mcs, mouth of coronary sinus. Bottom right, A subcostal left oblique view even further to the left shows the cephalad septum secundum (closed arrowheads) and the septum primum (open arrowheads) to be intact. (The cephalad septum secundum is absent in sinus venosus defect of the superior vena cava type.)

REFERENCES

1. Mantini E, Grondin GM, Lillehei CW, Edwards JE. Congenital anomalies involving the coronary sinus. Circulation 1966; 33:317-27. 2. Yeager SB, Chin AJ, Sanders SP. Subxiphiod two-dimensional echocardiographic diagnosis of coronary sinus septal defects in children. Am J Cardiol 1984;54:686-7. 3. Kurosawa H, Yagi Y, lmanura E, Koyanagi H, Satomi M, Nakazawa M, Takao A. A problem in Fontan’s operation: sinus septal defect complicating tricuspid atresia. Heart Vessels 1985;1:48-50. 4. Rumisek JD, Pigott ,JD, Weinberg PM, Norwood WI. Coronary sinus septal defect associated with tricuspid atresia. J Thorac Cardiovasc Surg 1986;92:142-5. 5. Ross DN. The sinus venosus type of atria1 septal defect. Guys Hosp Rep 1956;105:376-81. 6. McCormack RJM, Pickering D, Smith II. A rare type of atria1 septal defect. Thorax 1968;23:350-2. 7. Freedom RM. Culham JAG. Rowe RD. Left atria1 to coronarv sinus fenes&ion (partially unroofed coronary sinus). Bi Heart J 1981;46:63-8. 8. AIboliras ET, Chin AJ, Barber G, Helton JG, Pigott JD. Detection of aorta-pulmonary window by pulsed and color Doppler echocardiography. AM HEART J 1988;115:900-2.

Transesophageal echocardiographic diagnosis of coronary sinus type atrial septal defect Yasushi Sunaga, MD, Kayo Hayashi, MD, Naohiko Okubo, MD, Yoshio Taniichi, MD, Tetsuro Sugiura, MD, Nobuyuki Tsuda, MD, Toshiji Iwasaka, MD, and Mitsuo Inada, MD Osaka, Japan Coronary sinus type atria1 septal defect is a rare cardiac malformation and is usually diagnosed during a surgical procedure or at autopsy. l, ’ This report describes a case of From the Second Department of Internal Medicine, Kansai Medical University, Osaka, Japan. Reprint requests: Yasushi Sunaga, MD, Second Department of Internal Medicine, Kansai Medical University, 1 Fumizono-cho. Moriguchi City, Osaka 570 Japan. 414141347

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American

December 1992 Heart Journal

Fig. 1. A, TTE imageshowing enlarged coronary sinus and a considerably increasedflow from the coronary sinusto the right atrium; B, longitudinal TEE imageobtained from the left sideof the heart revealing enlargedcoronary sinuswith no defect in its roof; C, scanningto the right, interatrial septal defect (arrow head) wasdemonstrated by TEE in the vicinity of the coronary sinusostium; D, a shunt flow from the left atrium to the right atrium was clearly observedon a TEE color flow image. CS, Coronary sinus;RA, right atrium; RV, right ventricle; LA, left atrium; MV, mitral valve; LV, left ventricle.

coronary sinustype atria1 septal defect that wasdiagnosed beforesurgery by transesophageal echocardiography(TEE). A 53-year-old man was admitted to our hospital for evaluation of arrhythmia and cardiomegaly. On auscultation, fixed splitting of the secondheart sound and a grade Z/6 diastolic murmur were audible at the secondleft intercostal spacenear the sternum. The chest roentgenogram showeda cardiothoracic ratio of 0.57 and increasedpulmonary flow. An ECG showed atria1 fibrillation and incomplete right bundle branch block. Transthoracic echocardiography (TTE) revealed right ventricular enlargementand paradoxical septal motion that was consistent with right ventricular volume overload. A defect of the interatrial septum was not found on the parasternal four-chamber view. By moving the transducer slightly inward from parasternalfour-chamber view, color flow Doppler imaging showed enlarged coronary sinus and a considerably increasedflow from the coronary sinus to the right atrium (Fig. 1, A). After injection of contrast agent into the left mediancubital vein, the right atrium wasopacified, and no contrast medium appeared in t,he coronary sinus.Further

study was done by TEE, which confirmed that all of the pulmonary veins drained into the left atrium. A longitudinal image, which was obtained from the left side of the heart, revealed enlargedcoronary sinuswith no defect in its roof (Fig. 1, B). When scanningto the right wasperformed, interatrial septaldefect wasdemonstratedin the vicinity of the coronary sinusostium (Fig. 1, C), and a shunt flow was clearly observed(Fig. 1, D). At operation, a 2 X 2 cm defect was found at the interatrial septum and was closedwith polytetrafluoroethylene membrane.* This left the coronary sinusflow draining into the right atrium. Coronary sinustype atria1 septal defect, which wasfirst described by Raghib et a1.,3 is caused by incomplete formation of the left atriovenous fold. Several subtypes of Raghib’sanomaly have beenreported asa result of various degreesof left atriovenous fold formation.2*4The type that wasfound in our casewas causedby incomplete fusion of the atria1 septum and atriovenous fold without persistent *Gore-tex vascular ates, Elkton. Md.

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left superior vena cava. Only a few cases of coronary sinus type atrial septal defect have been diagnosed before surgery by detecting the defect of the septum from various views and identifying a shunt flow from the left atrium to the right atrium by means of TTE.5~6 Because coronary sinus type atria1 septal defect is difficult to diagnose by routine TTE, the presence of a coronary sinus type atria1 septal defect must be inferred from identification of dilated coronary sinus, increased flow from coronary sinus, and right ventricular volume overload. However, dilated coronary sinus can be caused by persistent left superior vena cava or anomalous pulmonary venous return draining into coronary sinus. In this case, size and location of atria1 septal defect and the presence of roof in the coronary sinus were confirmed by the longitudinal scan of TEE. The absence of anomalous pulmonary venous return was confirmed by demonstration that all pulmonary veins were draining into the left atrium, and the diagnosis of persistent left superior vena cava was excluded by contrast echocardiography. To the best of our knowledge, this is one of the first reported cases of coronary sinus type atrial septal defect that was diagnosed before surgery and demonstrates the usefuIness of TEE in the identification of this defect. REFERENCES

1. GoorDA, LilleheiCW.Congenital malformations of theheart: 2. 3.

4.

5

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embryology,anatomy, and operative considerations.New York: Grune& Stratton, 1975:103-11. Lee ME, Sade RM. Coronarysinusseptaldefect:surgicalconsiderations. J Thorac CardiovascSurg197$78:563-g. RaghibG,RuttenbergHD, AndersonRC, AmplatzK, Adams P Jr, EdwardsJE. Terminationof left superiorvenacavain left atrium, atria1 septaldefect,andabsence of coronarysinus: a developmental complex. Circulation 1965;31:906-18. Quaegebeur J. Kirklin JW. Pacific0 AD. Baraeron LM Jr. &rgi&l experience with unroofed coronary sir&s. Ann Thorat Surg 1979;27:418-25. Takahashi H, Sakamoto T, Amano K, Hada Y, Serizawa T, Tomaru T, Kawauchi M, Furuta N, Furuse A, Asano K. Coronary sinustype atrial septaldefectdiagnosed by two-dimensional color Doppler echocardiography: a case report. J Cardiog 1985;15:1283-91. Konstantindes S, Van Tournout FAM, Bennett JM. Unroofed

coronarysinusassociated with atrial septaldefectandsignificant mitral regurgitation. 99:377-g.

J Thorac Cardiovasc Surg 1990;

Aneurysm of the noncoronary sinus of Valsalva ruptured into the left atrium Laure Cabanes,MD, Elias Garcia, MD, Christian VanDamme, MD, Alain Berrebi, MD, Patrick Donzeau-Gouge, MD, Jean Fouchard, MD, and Franqois Guerin, MD Paris, France

Aneurysms of the sinus of Valsalva are rare and usually rupture into the right chambersof the heart. We report a From the Department of Cardiology, Hopital Cochin, Rene DescartesUniversity. Reprint requests: Laure Cabanes, MD, Service de Cardiologie Hdpital Cochin, 27 Rue Saint Jacques, 75014 Paris, France. 4/4/41346

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caseof an aneurysm of the noncoronary sinusof Valsalva that ruptured into the left atrium. Diagnosiswas established before surgery by transesophagealechocardiography with color flow Doppler imaging. A 75-year-old woman was admitted to the hospital for treatment of severeexertional dyspnea. On examination, there wasa grade 4/6 continuous murmur, which washeard at the apex and radiating to the left axilla. Systolic and diastolic blood pressure were 140 and 40 mm Hg, respectively, and an increasedarterial pulsewasnoted. Moderate symptoms of congestive heart failure were present. ECG was normal, and a chest roentgenogram revealed a mild enlargement of the heart with evidence of pulmonary venous hypertension. Transthoracic echocardiography (Advanced Technology Laboratories, Inc., Bothell, Wash.), with a 2.25MHz transducer demonstrateda slight enlargement of the left ventricle with a good systolic contraction, (shorteningfraction, 35%). The left atrium wasdilated (45 mm in diameter). The aortic valve appearedto be normal. The aneurysm of the noncoronary sinus of Valsalva was detected in the parasternal and apical views, and a possible rupture was suspectedbecauseof a high-velocity flow, which was recorded throughout cardiac cycle in the left atrium with continuous wave Doppler echocardiography. The exact origin of this flow could not be identified with either pulsedwave or color flow Doppler echocardiography. Monoplane transesophagealechocardiography was performed with the sameequipment a 5 MHz transducer was used; a prominent deformity of the noncoronary sinusof Valsalva of 50 mm in diameter, which protruded into the left atrium was identified (Fig. 1). Furthermore, transesophagealechocardiographyclearly demonstrated a 7 mm dehiscenceof the wall of the aneurysm (Fig. 1) and color flow Doppler imaging showed a continuous mosaic jet, which flowed from the sinus and extended into the left atrium through this dehiscence(Fig. 2). Color flow Doppler imaging allowed direct visualization of systolic and diastolic filling of the aneurysm.There wasno evidence of any other abnormality commonly associatedwith aneurysmsof the sinus of Valsalva. The aortic leaflets appeared to be normal with no aortic regurgitation; there wasno ventricular septal defect, and coronary arteries were normal during the preoperative cardiac catheterization. The patient underwent a cardiopulmonary bypass:the aortic valve and the left and right coronary sinuswere normal. Elective repair of the posterior sinuswasundertaken: the defect was sutured, and the posterior sinus wasreconstructed with a Geseal’spatch (Vascutek Ltd., Renfrewshire, Scotland). The postoperative coursewas uneventful. Sinusof Valsalva aneurysmsarisefrom the noncoronary sinusin about 25% of casesand rupture more frequently into the right atrium or ventric1e.l However, perforation may occur in the left ventricle, the interventricular septum, a pulmonary artery, the superior vena cava, the pericardium, or the pleura. To our knowledge,this report may be the first oneto describea rupture of the noncoronary sinus of Valsalva aneurysm into the left atrium. Diagnosiswas established before surgery with transthorathoracic and transesophagealechocardiography. Previous reports outlined the usefulnessof Doppler echocardiography in the assessmentand managementof sinus of Valsalva aneu-