PII S0003 4975(00)02244 X same as those in the adult population Myocyte mitotic activity is thought to persist for 3 to 6 months after birth 8, conveying the potential for myocyte division, growth,.PII S0003 4975(00)02244 X same as those in the adult population Myocyte mitotic activity is thought to persist for 3 to 6 months after birth 8, conveying the potential for myocyte division, growth,.
Ann Thorac Surg 2001;71:719 –22 CASE REPORT MORALES ET AL LEFT ATRIAL ANEURYSM 719 ventriculectomy for dilated cardiomyopathy in a newborn J Thorac Cardiovasc Surg 1999;117:1017– McCarthy PM, Starling RC, Wong J, et al Early results with partial left ventriculectomy J Thorac Cardiovasc Surg 1997; 114:755– 65 Limas CJ, Limas C DNA polymerases during postnatal myocardial development Nature 1978;271:781–2 Left Atrial Aneurysm J Mark Morales, MD, Sanjeet G Patel, Joseph H Jackson, MD, James A Duff, MD, and James W Simpson, MD Driscoll Children’s Hospital, Corpus Christi, Texas Aneurysms of the left atrium are rare abnormalities They can be congenital or acquired Whereas a true congenital aneurysm presents as isolated pathology, inflammatory or degenerative processes involving the endocardium are associated with the acquired type The clinical records of patients with the diagnosis of left atrial aneurysm were reviewed, along with the surgical strategies, current literature, and patient outcomes Because of the risk of lifethreatening complications, surgery is recommended even in asymptomatic cases Resection and mitral valvuloplasty should be the treatment of choice (Ann Thorac Surg 2001;71:719 –22) © 2001 by The Society of Thoracic Surgeons Fig Partial left ventriculectomy (A) At resection, the endocardium showed extensive fibroelastosis (B) Surgically remodeled left ventricle same as those in the adult population Myocyte mitotic activity is thought to persist for to months after birth [8], conveying the potential for myocyte division, growth, and extensive cardiac remodeling in infancy I solated aneurysm of the left or right atrium is a rare congenital abnormality, first described by Semans and Taussig [1] in 1938 There have been isolated reports in the surgical literature; the age of presentation varies widely, from month to 66 years The appearance of an aneurysm as an isolated pathology, without evidence of predisposing, inflammatory, or degenerative processes, indicates a congenital origin Acquired aneurysmal dilatation of the left atrium is seen secondary to inflammatory processes References Case Reports Vouhe´ PR, Baillot-Vernant F, Trinquet F, et al Anomalous left coronary artery from the pulmonary artery in infants: which operation? When? J Thorac Cardiovasc Surg 1987;94:192–9 Del Nido PJ, Duncan BW, Mayer JE Jr, Wessel DL, La Pierre R, Jonas RA Left ventricular assist device improves survival in children with left ventricular dysfunction after repair of anomalous origin of the left coronary artery from the pulmonary artery Ann Thorac Surg 1999;67:169–72 Batista RJV, Santos JLV, Takeshita N, Bocchino L, Lima PN, Cunha MA Partial left ventriculectomy to improve left ventricular function in end-stage heart disease J Card Surg 1996; 11:96–7 Katsumata T, Westaby S Left ventricular reduction operation in ischaemic cardiomyopathy: a note of caution Ann Thorac Surg 1997;64:1154– Yoshii S, Hosaka S, Takahashi W, et al Partial left ventriculectomy in an infant with dilated cardiomyopathy J Thorac Cardiovasc Surg 1999;117:616– Berger S, Tweddell JS, Frommelt PC, Weinhaus L Partial left Patient © 2001 by The Society of Thoracic Surgeons Published by Elsevier Science Inc An 11-month-old Hispanic boy, born full term, 10 lbs weight at birth, sustained a cardiorespiratory arrest shortly after birth He was resuscitated and placed on mechanical ventilation, and after several weeks of hospitalization he was discharged home At the age of month he was found to have a murmur; 11 months later he was transferred to our institution exhibiting cardiomegaly Chest auscultation demonstrated 2/6 holosystolic murmur throughout the precordium, hepatomegaly, and bibasilar rales Chest roentgenogram showed significant Accepted for publication March 31, 2000 Address reprint requests to Dr Morales, PO Box 30104, Corpus Christi, TX 78463-0104; e-mail: moralej@driscollchildrens.org 0003-4975/01/$20.00 PII S0003-4975(00)02244-X 720 CASE REPORT MORALES ET AL LEFT ATRIAL ANEURYSM Fig Magnetic resonance imaging scan revealing large left atrial aneurysm displacing the heart anteriorly cardiomegaly Magnetic resonance imaging of the chest demonstrated a large mass displacing the heart (Fig 1) An echocardiogram demonstrated mitral insufficiency and a giant left atrium Cardiac catheterization revealed a large aneurysm of the left atrium (Fig 2) At the time of surgery, through a median sternotomy, on cardiopulmonary bypass, moderate hypothermia, and aortic cross-clamp, the heart was displaced superiorly and anteriorly, and the posterior aspect of the heart was exposed, revealing a large aneurysm of the left atrium that projected posteroinferiorly, displacing the left ventricle anteriorly The neck of the sac extended to the level of the posteromedial commissure without directly involving it The aneurysmal sac was resected to the base of the commissure and closed with a double running layer of 5-0 Prolene A standard atriotomy was performed, and the valve was tested, yielding no significant insufficiency The patient was asymptomatic at the 2-year follow-up Pathologic examination demonstrated a true aneurysmal sac of by 4.2 cm, with endocardial lining All layers of the atrial wall were present, indicating a true atrial aneurysm (Fig 3) Fig Frame of cardiac catheterization on LAO projection showing left atrial aneurysm Ann Thorac Surg 2001;71:719 –22 Fig Histologic section of left atrial aneurysm wall (ϫ50) The top section shows the endocardial surface, the middle area is collagenous atrial substance, and the lower section shows the cardiac atrial muscle Patient This child is a 7-year-old African American with a medical history significant for cardiomegaly and congestive heart failure Positive clinical findings included a holosystolic murmur with a diastolic rumble, hepatomegaly, and cardiac cachexia The child was evaluated in the clinic, where the echocardiogram demonstrated severe mitral insufficiency, mild mitral stenosis, and an aneurysmal left atrium Chest roentgenogram showed significant cardiomegaly; electrocardiogram displayed left atrial hypertrophy and left ventricular enlargement The patient was scheduled for elective mitral valve repair and aneurysmal excision A week later he decompensated, requiring hospitalization Cardiac catheterization demonstrated moderate to severe mitral regurgitation, with mild mitral stenosis along with a large left atrial aneurysm At surgery, through a median sternotomy under cardiopulmonary bypass, the left atrium was approached through a standard incision The aneurysm was identified extending posteriorly and involving the posteromedial commissure of the mitral valve (Fig 4) The valve leaflets and the subvalvular apparatus were fibrosed The aneurysm was repaired from the inside of the left atrium Fig Involvement of the posterolateral commissure by the atrial aneurysm Ann Thorac Surg 2001;71:719 –22 by excising the abnormal sac and approximating the fibrous neck with a double layer of 5-0 Prolene, and the appendage was plicated A valvuloplasty was attempted but unfortunately proved to be unsuccessful The mitral valve was replaced with a 3l-mm Carbomedics mechanical prosthesis The pathologic section demonstrated myxomatous changes in the valve and endocardium of the left atrium Postoperatively, the patient is doing well, remaining in junctional rhythm year after surgery Comment Congenital aneurysm of the left atrium is a rare condition that usually presents as an isolated roentgenogram finding or with symptoms related to cardiac arrhythmia and systemic embolism The mean age of presentation in a previously reported review was 23.5 years [3] Although there is some discrepancy in the literature in regards to what constitutes a congenital aneurysm and which aneurysmal dilatations of the left atrium are secondary to congenital mitral regurgitation, this difference carries only academic value, because the clinical approach is the same: resection of the aneurysm and repair or replacement of the valve as needed [4, 5, 6] Left atrial aneurysms are most commonly found in the left atrial appendage, but they can present in the atrial wall, as was the case in both of our patients Such aneurysms can be found with an intact pericardium [6] or with a pericardial defect [7], in which case the aneurysmal protrusions resemble dog’s ears [8] The aneurysms can be classified as congenital or acquired, the latter being more common and associated with conditions such as rheumatic heart disease, tuberculosis, or syphilitic myocarditis The absence of anatomic abnormalities of the mitral valve as well as the lack of degenerative or inflammatory processes in the histologic section support a congenital etiology Mitral regurgitation can occur primarily or as a consequence of the atrial enlargement and the asymmetric annular deformity Whereas older patients are usually asymptomatic, newborns and infants present with cardiac decompensation [4, 9] Congestive heart failure may be related to an anatomic obstruction of the pulmonary venous drainage [4] Respiratory distress, especially in the neonate, can be secondary to displacement of the relatively flexible mediastinum and airway obstruction (as one may observe in Fig 1) Cardiac tamponade due to the limitation of diastolic expansion of the left ventricle has been described in the literature [9] Other symptoms, prevalent in the older patients, include systemic embolization or supraventricular arrhythmias, which probably result from an abnormal atrial wall [10] In asymptomatic patients, the diagnosis can be suspected by the enlarged heart silhouette of the chest roentgenogram A mediastinal mass can be ruled out by computed tomography of the chest or magnetic resonance imaging Transthoracic echocardiogram may reveal the enlarged left atrium and mitral insufficiency; however, the point of communication of the aneurysm with the left atrium can be difficult to image A transesophageal echocardiogram is much better for evaluating this area of the heart; moreover, CASE REPORT MORALES ET AL LEFT ATRIAL ANEURYSM 721 the atrial appendage can be imaged for the presence of thrombus [11] Foale and colleagues [12] characterized the echocardiographic findings of left atrial aneurysms as: (1) origin from an otherwise normal left atrium, (2) a welldefined communication with the atrium, (3) a position within the pericardium, and (4) distortion of the left ventricular free wall by the aneurysm Cardiac catheterization with cineangiocardiography is probably still recommended in order to evaluate pressures, shunts, and biopsy tumors if necessary Because of the risk of life-threatening complications, including tamponade (patient 1), tachyarrhythmia, systemic embolization, congestive heart failure, or the potential theoretical risk of rupture, surgery is recommended even in asymptomatic cases The principles of surgery should emphasize minimal manipulation of the aneurysm until cardiopulmonary bypass is established, in order to avoid embolization, especially if a transesophageal echocardiogram has shown thrombus within the heart In neonates or infants, the aneurysm is best approached posteriorly after displacing the apex of the heart superiorly The aneurysmal sac is resected to the neck, and the left atrium is closed The mitral valve can be tested with cold saline infusion through a standard left atriotomy, or by placing a vent in the apex of the left ventricle In older patients, aneurysms can be approached through the left atrium, and the sac can be resected and the valve repaired or replaced as needed It is important to assess the relationship of the neck of the aneurysm with the posterolateral mitral commissure, because on occasion the aneurysm extends into the commissure, requiring a partial annuloplasty (De Vega) in order to reestablish mitral competence Aneurysms involving the atrial appendage can be resected by a left thoracotomy without the use of cardiopulmonary support [12] In conclusion, early diagnosis and prompt surgical therapy will obviate the risk of devastating complications of this rare problem References Semans JH, Taussig HB Congenital aneurysmal dilatation of the left auricle Bull Johns Hopkins Hosp 1938;63:404 Hougen TJ, Mulder DG, Gyepes MT, Moss AJ Aneurysm of the left atrium Am J Cardiol 1974;33:557– 61 Shaher RM, Anis W, Alley R, Mentzer J Congenital enlargement of the left atrium J Thorac Cardiovasc Surg 1972;63: 292 Stone KS, Brown JW, Canal D, Caldwell R, Hurwitz R, King II Congenital aneurysm of the left atrial wall in infancy Ann Thorac Surg 1990;49:476– Grinfeld R, Trainini JC, Roncoroni A, Fabrykant F, Cacheda H, Tripodi G Congenital Aneurysm of the left atrium Ann Thorac Surg 1985;39:469–78 Grinfeld R Congenital left atrial aneurysm [letters to the editor, reply] Ann Thorac Surg 1986;41:581– Fry W Herniation of the left auricle Am J Surg 1956;86:736 Palacio J, Guido JJ, Noda VN, et al Megaorejuela izquierda congenital Prensa Medica Argentina 1960;47:1505 Dimond EG, Kittle CF, Voth DW Extreme hypertrophy of the left atrial appendage: the case of a giant dog ear Am J Cardiol 1960;5:122–5 722 CASE REPORT ITO ET AL CONGENITAL PULMONARY VEIN STENOSIS 10 Morrow AG, Behrendt DM Congenital aneurysm (diverticulum) of the right atrium Circulation 1968;38:124– 11 Comess KA, Labate DP, Winter JA, Miller DC Congenital left atrial appendage aneurysm with intact pericardium: diagnosis by transesophageal ecliocardiography Am Heart J 1990;120:993–5 12 Foale RA, Gibson TC, Guyer DE, Gillam L, King ME, Weyman AE Congenital aneurysms of the left atrium: recognition by cross-sectional echocardiography Circulation 1982;66:1065–9 13 Ganeshakrishan KI, Khandeparkar JMS, Natrajan VM, Agrawal NB, Oswal DH, Magotra RA Thorac Cardiovasc Surg 1992;40:382– Congenital Pulmonary Vein Stenosis Associated With Cor Triatriatum Masayoshi Ito, MD, Seiya Kikuchi, MD, Yoshikazu Hachiro, MD, and Tomio Abe, MD Department of Thoracic and Cardiovascular Surgery, Sapporo Medical University School of Medicine, Sapporo, Japan We report an 11-year-old boy who underwent surgical correction for stenosis of the right and left lower pulmonary veins at their junction with the left atrium and associated cor triatriatum The embryology and therapeutic approaches to congenital pulmonary veins stenosis are discussed (Ann Thorac Surg 2001;71:722–3) © 2001 by The Society of Thoracic Surgeons C ongenital pulmonary veins (PV) stenosis is a rare anomaly Edwards [1] found patients (0.4%) among 711 patients with major cardiac and thoracic vascular anomalies Congenital PV stenosis is often an isolated lesion, but it may coexist with total anomalous pulmonary vein connection, complete transposition of the great artery, ventricular septal defect, and other anomalies [2] Despite its embryologic relation [3], congenital PV stenosis associated with cor triatriatum is extremely rare We present such a rare case of PV stenosis associated with cor triatriatum and discuss the embryology and therapeutic approaches to congenital PV stenosis The patient, a 2-month-old male infant, was taken to another hospital with tachypnea He was diagnosed with complex cor triatriatum (Lucus-Schmit type IB1, Niwayama type IIA1), and was immediately operated on to remove the abnormal septum between the common pulmonary venous chamber (CPVC) and the left atrium Accepted for publication April 13, 2000 Address reprints requests to Dr Ito, Department of Thoracic and Cardiovascular Surgery, Sapporo Medical University School of Medicine, South 1, West 16, Chuou-ku, Sapporo 060-8543, Japan; e-mail: shinwa@seagreen ne.jp © 2001 by The Society of Thoracic Surgeons Published by Elsevier Science Inc Ann Thorac Surg 2001;71:722–3 and to close the septal defect between the CPVC and the right atrium His condition improved in the weeks after the operation, and postoperative cardiac catheterization revealed a satisfactory result He continued to be healthy and without limitation to activities until 11 years of age when he presented with a moderate pulmonary hypertension and recurrence of cor triatriatum by the cardiac echocardiogram He was referred to our hospital Physical examination on admission showed a well-developed boy (weight, 40.5 kg; height, 157.5 cm) The heart sounds were almost normal Chest roentgenogram showed a slightly prominent main pulmonary artery segment; the cardiothoracic ratio was 51% Preoperative cardiac catheterization showed moderate pulmonary hypertension (35 mm Hg), and a pressure gradient between pulmonary wedge pressure and left atrium pressure (11 mm Hg) He was diagnosed with a recurrence of cor triatriatum and was referred to our department for the possibility of a surgical repair At this point, the pulmonary vein stenosis was not recognized He underwent a repair of the recurrent cor triatriatum After instituting cardiopulmonary bypass using direct cannulation of the superior and inferior venae cava, moderate hypothermia was achieved The aorta was cross-clamped and cold blood cardioplegia was administered through the aortic root The CPVC was opened through a vertical incision anterior to the right PVs An abnormal septum was noted between the CPVC and the left atrium, with a hole of about cm A membranous diaphragm obstructed the entrances of the right and left lower PVs into the CPVC, and the openings were about mm and mm, respectively The right and left upper PVs were connected with the CPVC without stenosis The abnormal septum was excised to make an opening as large as possible The membranous diaphragm at the junction of the right and left lower PVs and the CPVC was then removed Post repair cardiac function was good and after cardiopulmonary bypass was discontinued, the pulmonary pressure was 20/14 mm Hg, whereas pressure in the radial artery was 110/62 The postoperative course was uneventful The postoperative cardiac echocardiogram and cardiac catheterization revealed satisfactory results The patient was discharged from the hospital 13 days after the operation and at present (ie, months after surgery), he is doing well Histologically, the membranous diaphragm removed from the orifices of the PVs showed intimal fibrous thickening and there was no cell infiltration suggestive of active inflammation Comment Congenital PV stenosis has an embryologic relation with total anomalous pulmonary vein connection and cor triatriatum [3] By the mm stage, a diverticulum has formed in the future left atrial portion of the sinoatrial region The diverticulum, or common PV, grows toward the developing lungs and ultimately connects with the PVs, which have already formed When this drainage pathway is established, the early connections between 0003-4975/01/$20.00 PII S0003-4975(00)02346-8 ... showing left atrial aneurysm Ann Thorac Surg 2001;71:719 –22 Fig Histologic section of left atrial aneurysm wall (ϫ50) The top section shows the endocardial surface, the middle area is collagenous atrial. .. resection of the aneurysm and repair or replacement of the valve as needed [4, 5, 6] Left atrial aneurysms are most commonly found in the left atrial appendage, but they can present in the atrial wall,... ET AL LEFT ATRIAL ANEURYSM 721 the atrial appendage can be imaged for the presence of thrombus [11] Foale and colleagues [12] characterized the echocardiographic findings of left atrial aneurysms