Anatomic and functional "obstruction" of the outflow tract in atrioventricular septal defects with separate valve orifices ("ostium primum atrial septal defect"): an echocardiographic study

Left ventricular (LV) outflow tract (OT) obstruction can be treacherous in any form of atrioventricular (AV) septal defect. The properties of the LVOT were investigated echocardiographically in 64 patients with separate valve orifices ("ostium primum atrial septal defect") who had survived corrective surgery. M-mode and cross-sectional echocardiographic (echo) images were made of the LVOT. The degree of malalignment of the aorta with the ventricular septum, the left atrium-aortic ratio, the fractional LV shortening and the diameter of the LVOT were recorded. Fixed anatomical obstruction was found in 3 patients, consisting of muscular bands or abnormal attachment of tension apparatus. Malalignment of the aorta with the ventricular septum was found in 62% of the patients. The diameter of the LVOT was smaller than that of the aortic root in 71% of the cases. The mean diameter of the LVOT was 92 +/- 27% (range 35 to 143%) of the aortic root diameter. Because its walls are mainly muscular, the LVOT constricts during systole. The mean end-systolic diameter of the LVOT was 77 +/- 22% (range 23 to 129%) of the aortic root diameter. Sequential measurements showed that the LVOT constricted gradually, but the velocity of constriction in patients with the most severe narrowing showed a distinct maximum in the first fifth of systole. In conclusion, a series of elements contribute to a potentially perilous arrangement of the LVOT in patients with AV septal defect. This intrinsically narrow tunnel was constricted during systole by its muscular walls.(ABSTRACT TRUNCATED AT 250 WORDS)     

Long-term follow-up after surgical repair of ostium primum atrial septal defect in adults

Objectives. We sought to determine the long-term survival rates and defect-related morbidity of adult patients who undergo surgical repair of an ostium primum atrial septal defect.Background. The natural history of patients undergoing such surgical repair in adulthood remains unclear.Methods. We followed up 33 patients who underwent surgical correction of an ostium primum atrial septal defect at our institution at a mean age of 42 years (range 20 to 73); 12 of these patients were >50 years old at the time of operation. Four patients had moderate preoperative exercise incapacity (New York Heart Association functional class >11) and six had atrial fibrillation. Nine and four patients, respectively, had a preoperative mean pulmonary artery pressure >25 mm Hg or pulmonary vascular resistance >4 Wood U. Autologous pericardium was used to patch the defect in 30 patients (91%). Mitral valvuloplasty, consisting of cleft repair (n=10), and mitral valve replacement (n = 2) were performed selectively.Results. There were no operative deaths. At a mean follow-up interval of 5.3 years (range 1 to 18.2), all 28 surviving patients are free of exercise limitation (functional class 1). Late postoperative deaths occurred in five patients (15%) and were related to myocardial infarction, stroke, hepatic failure, renal failure or sepsis. Reoperation within the 1st postoperative year was required in two patients (6%) because of a residual ostium primum defect in one and severe mitral regurgitation in the other. The presence of advanced age at operation, symptoms, atrial arrhythmias, mitral regurgitation or moderately increased pulmonary vascular resistance did not predict late postoperative mortality, complications or functional capacity.Conclusions. An ostium primum defect can be repaired in adult patients with the expectation of excellent long-term results, independent of age at operation and preoperative mitral valve function and despite the presence of atrial fibrillation or moderately elevated pulmonary vascular resistance.     

Aortic valve insufficiency: an unrecognized complication of the surgical repair of ostium primum atrial septal defect

Between 1974 and 1989, eight children undergoing surgical repair of a partial atrioventricular canal comprising an ostium primum atrial septal defect, mitral regurgitation due to a cleft mitral valve (6 cases) and a small ventricular septal defect (1 case) developed aortic regurgitation. This complication was diagnosed on the finding of a diastolic murmur 1 day to 9 years after the repair of the atrial septal defect. The degree of regurgitation was Grade I in 4 cases but in the other patients it was severe from the outset requiring emergency surgery the next day (1 case), or moderate at the beginning but rapidly progressive (1 case) or slowly progressive (2 cases) leading to aortic valvuloplasty in these 3 patients. The cause of the regurgitation was iatrogenic: perforation of the non coronary cusp secondary to repair of the ostium primum atrial septal defect. None of the patients required aortic valve replacement. After surgical valvuloplasty, all children had Grade 1 aortic regurgitation. Color coded Doppler echocardiography confirmed the diagnosis and helped assess the clinical course of this lesion during follow-up.     

Results of surgical repair of ostium primum atrial septal defect in adult patients

BACKGROUND AND AIM OF THE STUDY: Ostium primum atrial septal defect (ASD I) is very rarely observed in the adults. Although ASD I was first corrected surgically almost 50 years ago, the efficacy of surgical treatment in adults has not been well documented. Thus, the long-term outcome of patients aged over 20 years and having surgical repair of ASD I was examined. METHODS: Fifteen patients (10 males, five females; mean age 31.4+/-13.1 years; range: 20 to 56 years) who had surgical repair of ASD I between 1982 and 2000 were followed. All patients were examined physically and underwent chest radiography, ECG and echocardiography (cross-sectional and Doppler) before and after surgery. Autologous pericardium was used to close the defect in 14 patients, and a direct suture was used in one patient. Mitral valvuloplasty (repair of the cleft) was performed in 12 patients, and De Vega annuloplasty in eight. RESULTS: There were no hospital deaths. In one case a pacemaker was implanted five days after surgery because of complete heart block. Preoperatively, nine patients (60%) were in NYHA classes III and IV; at the end of follow up, 14 (93.3%) were in classes I and II. Preoperatively, 13 patients had sinus rhythm, and one each had atrial fibrillation (AF) and rhythm from the atrioventricular sinus. During follow up, three patients developed AF which was treated successfully with electrical cardioversion. The preoperative mean cardiac volume index of 695 +/- 216 ml/m(2) was reduced significantly after repair to 523 +/- 108 ml/m(2) (p < 0.05). Before surgery, mitral regurgitation was observed (severe in five cases, moderate in seven, mild in three). Postoperatively, a residual intracardiac shunt was identified in one case. Postoperative mitral regurgitation was noted in six patients (moderate in two, mild in four). The right ventricular dimension was decreased significantly, from 5.0 +/- 1.5 mm before surgery to 3.2 +/- 0.6 mm after repair (p < 0.001). CONCLUSION: Patients aged over 20 years with ASD I benefit from surgical repair of the defect.     

Conduction abnormalities detected by electrophysiologic testing following repair of ostium primum atrioventricular septal defect

Since 1983 we have performed electrophysiologic studies in 6 patients who had previously undergone repair of an ostium primum atrioventricular septal defect. Information obtained during electrophysiologic studies was crucial in guiding appropriate pacemaker therapy in these patients. As judged from the resting electrocardiogram, sinus or junctional bradycardia was present in 3/6, atrial flutter / fibrillation in 2/6, and paced rhythm in 2 patients who had had ventricular pacemakers implanted for complete atrioventricular block. During maximal exercise testing 4 patients had reduced heart rates; 2 had sudden drops in heart rate at 1 min postexercise; 1 patient had exercise induced ventricular bigeminy; and 1 patient with atrial flutter and 2: 1-4: 1 block at rest developed 1: 1 conduction during Stage II with an effective ventricular rate of 220/min. During electrophysiologic studies, the maximum corrected sinus node recovery time was abnormal in five of the six, ranging from 410 to 5630 msec. There was no spontaneous atrial rhythm in the other patient. Complete atrioventricular block was present in 2 patients while the atrioventricular Wenckebach phenomenon occurred abnormally at atrial pacing cycle lengths greater than 450 msec in 2 others. Supraventricular tachycardia or atrial flutter/fibrillation, was either spontaneous or induced in 2/6 patients, while ventricular tachycardia was induced in 1/3 patients who underwent programmed ventricular stimulation. Electrophysiologic studies were important in unmasking severe sinus node disease in 3 patients and atrioventricular node disease in 2. We therefore recommend that electrophysiologic studies be strongly considered as part of the evaluation of conduction abnormalities following repair of ostium primum atrioventricular septal defect.     

Atrioventricular septal defect with a well developed primary component of the atrial septum ("septum primum")

A heart is described with an atrioventricular septal defect in which the lower part of the atrial septum ("septum primum") was well developed and in which superior and inferior bridging leaflets were attached neither to the crest of the ventricular septum nor the inferior rim of the atrial septum. Diagnostic pre-operative angiographic and echocardiographic features and the method of surgical correction are outlined.     

Angiocardiographic appearances of atrioventricular defects with particular reference to distinction of ostium primum atrial septal defect from common atrioventricular orifice

Preoperative distinction between common atrioventricular orifice and ostium primum atrial septal defect may be difficult. To improve diagnostic accuracy, the right and left ventricle angiocardiograms were reviewed 'blind' in 92 patients with atrioventricular defects. The true diagnosis was known from necropsy or surgery in 60. Angiocardiograms had been obtained in various projections with or without craniocaudal tilt. Those features thought to distinguish between common orifice and ostium primum were coded, together with the ventricular systolic pressures. Computerised disciminant function analysis identified the following distinguishing features: (1) right ventricular systolic pressure; (2) immediate right ventricular outflow tract opacification from the left ventricle; (3) identification of the anterior attachment of the mitral component; (4) recognition of a single straddling atrioventricular orifice; (5) passage of contrast medium above or below the anterior or posterior bridging leaflets. Feature (3) indicates that in contrast to classic teaching the direct septal attachment of the mitral component does not contribute to the 'gooseneck' in complete atrioventricular defects. The significance of (4) and (5) is that they may be identified from right as well as left ventriculography, and are more likely to be identified in oblique than standard projections. Computerisation produced a correct diagnosis in 92 per cent of known cases, and determined precise probabilities of diagnosis in the remainder.     

Masked subaortic stenosis in ostium primum atrial septal defect: Recognition and treatment

Five patients with ostium primum atrial septal defect (ASD) and a cleft mitral valve had no hemodynamic evidence of left ventricular (LV) outflow tract obstruction on preoperative cardiac catheterization. After surgical closure of the ASD and repair of the mitral cleft, all 5 patients manifested subaortic stenosis with pressure gradients ranging from 10 to 120 mm Hg. Postoperative LV angiograms revealed systolic narrowing of the outflow tract, and the same outflow tract dynamics were recognized on reviewing the preoperative angiograms and echocardiograms. Persistence or exaggeration of the characteristic diastolic “goose-neck” deformity during LV systole in atrioventricular canal defects is diagnostic of a potential or actual subaortic obstruction. This diagnostic sign is also readily recognizable by 2-dimensional echocardiography, and when present, the surgeon should be alerted to explore the LV outflow tract because the outflow tract anatomy is not readily apparent at operation aimed solely at closing the ASD and repairing the cleft mitral valve.     

Angiocardiographic appearances of atrioventricular defects with particular reference to distinction of ostium primum atrial septal defect from common atrioventricular orifice.

Preoperative distinction between common atrioventricular orifice and ostium primum atrial septal defect may be difficult. To improve diagnostic accuracy, the right and left ventricle angiocardiograms were reviewed ''blind'' in 92 patients with atrioventricular defects. The true diagnosis was known from necropsy or surgery in 60. Angiocardiograms had been obtained in various projections with or without craniocaudal tilt. Those features thought to distinguish between common orifice and ostium primum were coded, together with the ventricular systolic pressures. Computerised disciminant function analysis identified the following distinguishing features: (1) right ventricular systolic pressure; (2) immediate right ventricular outflow tract opacification from the left ventricle; (3) identification of the anterior attachment of the mitral component; (4) recognition of a single straddling atrioventricular orifice; (5) passage of contrast medium above or below the anterior or posterior bridging leaflets. Feature (3) indicates that in contrast to classic teaching the direct septal attachment of the mitral component does not contribute to the ''gooseneck'' in complete atrioventricular defects. The significance of (4) and (5) is that they may be identified from right as well as left ventriculography, and are more likely to be identified in oblique than standard projections. Computerisation produced a correct diagnosis in 92 per cent of known cases, and determined precise probabilities of diagnosis in the remainder.     

Platypnea-orthodeoxia in a patient with ostium primum atrial septal defect with normal right heart pressures

We describe an adult patient with an ostium primum atrial septal defect (ASD) and a patent foramen ovale (PFO) with normal right heart pressures who presented with platypnea and orthodeoxia. A dilated aortic root encroaching into the region of the ASD and PFO, along with a tricuspid regurgitant jet moving into the left atrium through the ASD found on the transesophageal echocardiogram may have been responsible for orthodeoxia. Surgical closure of these defects resulted in the disappearance of both platypnea and orthodeoxia.     

Are routine preoperative cardiac catheterization and angiography necessary before repair of ostium primum atrial septal defect?

Two-dimensional and Doppler echocardiography were compared with cardiac catheterization and angiography in the preoperative evaluation of ostium primum atrial septal defect. Preoperative echocardiographic examinations as well as operative reports of all patients (33 patients aged 2 months to 23 years at surgery) with ostium primum atrial septal defect or transitional atrioventricular (AV) canal defect having had echocardiography and surgical repair at The Children's Hospital, Boston from July 1983 to January 1986 were retrospectively reviewed. Original cardiac catheterization and angiographic reports also were reviewed. Preoperative echocardiography resulted in no false positive or false negative primary diagnoses when compared with the diagnoses obtained at preoperative angiography or surgery. Doppler assessment of mitral regurgitation correlated well with angiographic (93% agreement) and intraoperative (85% agreement) assessments of mitral regurgitation to within two diagnostic categories on the six level scoring system used. There was reasonably good agreement between the two-dimensional echocardiographic estimate of right ventricular systolic pressure and that measured at catheterization when expressed as percent of the simultaneous left ventricular pressure. Seven of nine ventricular septal defects observed intraoperatively were noted on preoperative echocardiography; five of these defects were detected on preoperative angiography. A variety of other surgically confirmed associated cardiovascular defects were observed by both preoperative techniques. However, echocardiography appeared to be superior to angiography for evaluation of AV valve morphology and papillary muscle architecture. This study implies that in children with typical clinical and two-dimensional echocardiographic and Doppler findings for ostium primum atrial septal defect or transitional AV canal defect, routine preoperative cardiac catheterization and angiography are unnecessary.     

Holt-Oram syndrome associated with combined ostium primum and secundum atrial septal defects: Successful surgical closure

A 55 year old man with the typical findings of Holt-Oram syndrome except for the presence of a septum primum defect in addition to the usual secundum defect is described. The clinical, physical, radiologic and operative findings are presented.     

Stenotic double-orifice mitral valve after surgical repaired partial atrioventricular septal defect

Double-orifice mitral valve (DOMV) is an uncommon congenital anomaly account for 1% of congenital heart disease. However, accurate diagnosis and evaluation of valve stenosis or regurgitation and other concomitant congenital anomalies due to DOMV are required to obtain suitable treatment. Two- and three-dimensional echocardiography can contribute valuable functional and anatomic information that can support to reach this goal. Here, we present a case of complete bridge-type DOMV that causes mitral stenosis after surgical repair of the partial atrioventricular septal defect in childhood.     

Adult patients after surgery of ostium primum type of atrial septal defects in childhood: echocardiography study

The authors examined, using transthoracic and transoesophageal echocardiography, 36 adult patients (15 men) aged 22 +/- 3.1 years (18-29 years) who were operated 12.2 +/- 3.7 years previously on account of a defect of the atrial septum type ostium primum. In these patients no other congenital cardiac defect was present. In addition to closure of the defect in the patients complete suture of the "cleft" of the anterior cusp of the mitral valve was performed, in 7 partial suture of the "cleft" of the anterior cusp of the mitral valve and in 4 commissuroplasty. In one instance later reoperation with replacement of the mitral valve by a mechanical prosthesis was performed. The control group was formed by 16 healthy volunteers (5 men) aged 22.1 +/- 3 years (19-31 years). Patients operated in childhood on account of an atrial defect of the ostium primum type have on echocardiographic examination, as compared with healthy volunteers, larger atria and the left ventricle, a thicker interventricular septum and left ventricular wall and a higher velocity of left ventricular filling during the late diastole. Higher values of parameters of the size and volume of the left ventricle are associated with the presence of mitral regurgitation. More marked changes of systolic or diastolic left ventricular function are not present, there are not even any echocardiographic signs of higher pressure in the atria and pulmonary artery. In none of the patients a residual shunt at the level of the atrial septum is present. Mitral regurgitation is found in two thirds of the patients, only in one case it was however moderately severe (grade 3). From the results it does not ensue which type of surgery of the "cleft" of the mitral valve has the best long-term results. In none of the patients tricuspid regurgitation of a higher grade than grade 1 is present.     

Echocardiographic evaluation and surgical implications of common atrioventricular canal defects with absent or diminutive ostium primum defect

Common atrioventricular canal defects without ostium primum defects are rare, and their accurate identification has important surgical implications. Retrospective echocardiographic database review identified subjects with common atrioventricular canal defects with absent or diminutive ostium primum defects. Surgical reports and initial and postoperative echocardiograms were reviewed to identify the imaging planes necessary to characterize this anomaly and the surgical challenges imposed by the diagnosis. Fourteen subjects were identified (93% with trisomy 21) with either absent (n = 6) or diminutive (n = 8) ostium primum defects. Malaligned conal septum was present in 50% of subjects with absent primum defects and 12.5% of subjects with diminutive defects. Immediate or long-term complications of the 11 postoperative patients included atrioventricular block (n = 4) and moderate (n = 5) or severe (n = 3) mitral regurgitation. In conclusion, echocardiographic features for the identification of common atrioventricular canal defects with absent or diminutive ostium primum defects are described. Surgical challenges involve attaining adequate exposure of the mitral component and achieving mitral valve competence.