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Biomedical subjects

J A van Son

Publications and source records attributed to J A van Son.

At least 19 recordsLinked to original sources
PubMed2001

Wilhelm Ebstein and Ebstein's malformation.

In 1866, Wilhelm Ebstein published a scholarly description of a tricuspid valve anomaly with dilation of the right atrium and patent foramen ovale that bears his name. However, his original report was almost overlooked. Despite a wide range of publications on the history of cardiac pathology and cardiac surgery, the international literature provides only scarce information regarding the personality of Wilhelm Ebstein and his original description of the anomaly that bears his name. In this article, we present biographical data of Wilhelm Ebstein and discuss how his original description of autopsy findings correlates with our current knowledge of this congenital disorder. It is the excellent correlation of Ebstein's pathologic findings with clinical notes of his colleague and Ebstein's hypotheses of the pathophysiology that made his publication a landmark in the description of a new entity. In addition, Ebstein's report provided a strong basis for the development of repair techniques for this rare anomaly 100 years later.

Ebstein Anomaly↗
PubMed2000

Urgency of operation in infracardiac total anomalous pulmonary venous connection.

BACKGROUND: Because the tendency for pulmonary venous obstruction in the infracardiac type of total anomalous pulmonary venous connection may be partially dependent on the connection of the descending vein to the portal vein, the inferior vena cava, or one of their tributary vessels, we reviewed our surgical experience with various subtypes of infracardiac total anomalous pulmonary venous connection. METHODS: The urgency of operation in 4 neonates with infracardiac total anomalous pulmonary venous connection was reviewed. RESULTS: Two patients with pulmonary venous obstruction in whom the descending vein connected to the portal vein were operated on immediately with successful outcome. One patient who had become critically ill after the ductus venosus had closed died before operation could be undertaken. One patient in whom the descending vein connected to the left hepatic vein was operated on electively with successful outcome. CONCLUSIONS: In hemodynamically stable patients with no clinical or echocardiographic signs of pulmonary venous obstruction, some form of differentiation with regard to urgency of operation may be appropriate. When the descending vein connects to the inferior vena cava or a hepatic vein, the operation may be performed on a semi-elective basis. In contrast, when the descending vein connects to the portal vein or the ductus venosus, operation should generally not be delayed because of the high likelihood of obstruction.

Emergencies↗
PubMed2000

Anatomical reconstruction of aorta and pulmonary trunk in patients with an aortopulmonary window.

We report a modified technique for repair of aortopulmonary window in which a native pulmonary artery flap is created and the aortopulmonary window is completely divided. The pulmonary artery flap is used to reconstruct the ascending aorta, and the defect in the pulmonary trunk is closed with an autologous pericardial patch. This repair avoids the use of a fabric patch with its inherent disadvantages and allows anatomical reconstruction of both the ascending aorta and the pulmonary trunk with low risk of early or late distortion of the aortic and pulmonary root structures.

Aorta↗
PubMed2000

Extracardiac conduit fontan procedure: early and intermediate results.

OBJECTIVE: The extracardiac Fontan procedure, as compared with classic atriopulmonary connections, may have the potential for optimizing ventricular and pulmonary vascular function by maximizing the laminar flow principle, by the avoidance of intra-atrial suture lines and cardiac manipulation, and by minimizing cardiopulmonary bypass time. In this study the clinical results of this procedure are assessed. METHODS: From January 1990 until January 1997, 45 patients (33 males and 12 females) with a median age of 4.0 years (range 2.7-38 years) underwent an extracardiac Fontan procedure for univentricular physiology. The underlying diagnoses included tricuspid atresia (n=19), double-inlet left ventricle (n=11), and complex anomalies (n=15). Forty patients (89%) were in sinus rhythm. The median ventricular ejection fraction was 60%. In 37 patients (82%) the procedure was staged. RESULTS: Median cardiopulmonary bypass time was 72 min, with a decrease to a median time of 24 min in the last ten patients. Aortic cross-clamping was avoided in 33 patients (73%). The intraoperative Fontan pressure and transpulmonary gradient were low: 13.6+/-3.2 and 8.5+/-3.9 mmHg, respectively. Transient supraventricular tachyarrhythmias were observed in six patients (13%). There was no early or late mortality. At a median follow-up of 64 months (range 26-105 months), 39 patients (87%) were in NYHA class I, four (9%) were in NYHA class II, and two (4%) were in class III. Forty patients (89%) remained in sinus rhythm. The median ventricular ejection fraction was 59%. The median arterial oxygen saturation raised from 82% preoperatively to 97%. Functional class (P=0.02), maintenance of sinus rhythm (P=0.04), and preservation of ventricular function (P=0.05) was superior in patients who were appropriately staged. None of the patients had atrial thrombus, chronic pleural effusions, or protein losing enteropathy. CONCLUSIONS: In the majority of patients, the extracardiac Fontan procedure, when performed as a staged procedure, provides excellent early and midterm results in terms of quality of life, maintenance of sinus rhythm, and preservation of ventricular function.

Adolescent↗
PubMed1999

Pulmonary artery sling: reimplantation versus antetracheal translocation.

BACKGROUND: We compared two repair techniques for pulmonary artery sling. The first comprised detachment of the aberrant left pulmonary artery from the right pulmonary artery and its implantation into the main pulmonary artery, and the second, translocation of the left pulmonary artery anterior to the trachea (without implanting it into the main pulmonary artery), resection of tracheal stenosis, and end-to-end reconstruction of the trachea. METHODS: Five symptomatic infants (3 boys and 2 girls; median age 5 months; range, 3 weeks to 11 months) with pulmonary artery sling were operated on through a median sternotomy with aid of cardiopulmonary bypass. In 3 patients, the left pulmonary artery was transected from the right pulmonary artery and implanted into the main pulmonary artery. In addition, the anterior trachea was augmented with a pericardial patch (n = 2). In the remaining 2 patients, associated tracheal stenosis was resected, the left pulmonary artery was translocated anterior to the trachea, and the trachea was reconstructed. RESULTS: All 5 infants survived the operation. The 3 patients in whom the left pulmonary artery was implanted into the main pulmonary artery had an uncomplicated postoperative course. All 3 patients, at a follow-up of 10 months to 7.9 years, were free of symptoms; the left pulmonary artery was documented to be widely patent. The remaining 2 patients in whom the left pulmonary artery was translocated anterior to the trachea could not be extubated. In both patients the distal trachea was compressed anteriorly by the left pulmonary artery. One of these patients died at 1 week postoperatively secondary to tracheal dehiscence. In the other patient, the left pulmonary artery was implanted into the main pulmonary artery with good result; at a follow-up of 3.9 years, mild residual stridor has persisted. CONCLUSIONS: In pulmonary artery sling, implantation of the aberrant left pulmonary artery into the main pulmonary artery, if necessary combined with anterior tracheoplasty, reliably eliminates tracheal and esophageal compression and maintains antegrade flow into the left pulmonary artery. Translocation of the left pulmonary artery anterior to the trachea without implanting it into the main pulmonary artery is not favored because that might result in anterior compression of the trachea. In addition, we are concerned about growth of the circumferential tracheal anastomosis in neonates and infants.

Female↗
PubMed1999

Repair of cor triatriatum associated with partially unroofed coronary sinus.

A young patient with cor triatriatum, secundum atrial septal defect, persistent left superior vena cava, partially unroofed coronary sinus, and moderate tricuspid regurgitation was documented to have both a left-to-right shunt from the common pulmonary venous chamber to the right atrium and a right-to-left shunt from the partially unroofed coronary sinus to the left atrium. Resection of the membrane dividing the left atrium, closure of the atrial septal defect and the partially unroofed coronary sinus with pericardial patches, and a tricuspid annuloplasty resulted in an excellent hemodynamic result.

Child↗
PubMed1999

Aortic root reconstruction with preservation of native aortic valve and sinuses in aortic root dilatation with aortic regurgitation.

BACKGROUND: We assessed the results of a modified technique for aortic root reconstruction including preservation of the native aortic valve and sinuses. METHODS: A modified technique for reconstruction of the aortic root was devised in which the native aortic sinuses are preserved and remodeled, the diameter of the sinotubular junction is reduced, the ventriculoaortic junction is reinforced with a Dacron prosthesis, and the coronary ostia are reimplanted. Since January 1995, this modified operative technique was performed in 13 patients with a mean age of 54 +/- 21 years. The median grade of aortic regurgitation was 3; in 10 patients it was caused by dilatation of the sinotubular junction, and 3 had additional annuloaortic ectasia. RESULTS: The aortic crossclamping time was 61 +/- 18 minutes. In-hospital mortality was 2 of 13 (15. 3%) patients, both deaths being related to complications of aortic dissection. In 1 patient aortic regurgitation increased to grade 3, necessitating aortic valve replacement. At a mean follow-up of 2.1 years, the remaining 10 patients had stable aortic valve function with a median grade of regurgitation of 1. The mean New York Heart Association functional class was 1.2. CONCLUSIONS: Aortic root reconstruction with preservation of the native aortic valve and sinuses allows symmetric reconstruction of the aortic sinuses and adaptation of the diameters of the sinotubular and ventriculoaortic junctions, thus optimizing aortic valve function. Moreover, it prevents contact of the aortic valve leaflets with the Dacron graft, which may enhance the durability of the repair.

Adolescent↗
PubMed1999

Conversion of atriopulmonary or lateral atrial tunnel cavopulmonary anastomosis to extracardiac conduit Fontan modification.

OBJECTIVE: Obstruction of the atriopulmonary anastomosis or the lateral atrial tunnel cavopulmonary anastomosis in the Fontan circulation for univentricular physiology may result in dilation of the right atrium or the right atrial free wall that is incorporated in the lateral atrial tunnel, respectively. Secondary detrimental sequelae may consist of supraventricular dysrhythmias, thromboembolism, right pulmonary vein compression, pleural effusions, and protein-losing enteropathy. Conversion of these Fontan connections to an extracardiac conduit cavopulmonary anastomosis may improve central systemic venous flow patterns and provide clinical improvement in these patients. METHODS: Eighteen patients (7-40 years old) with atriopulmonary anastomosis (n = 15) or obstructed lateral atrial tunnel cavopulmonary anastomosis (n = 3) presented at 5.7 +/- 3.9 years with moderate to severe right atrial dilation (n = 15), Fontan pathway obstruction (n = 12), atrial dysrhythmia (n = 13), pleural effusion (n = 8), right atrial thrombus (n = 3), right pulmonary vein compression (n = 3), and protein-losing enteropathy (n = 3). All patients underwent conversion to an extracardiac conduit cavopulmonary anastomosis. RESULTS: Two of the three patients with protein-losing enteropathy died (2/18; 11%) on the 30th and 52nd postoperative days. At a mean follow-up of 19 months, the remaining 16 patients had marked (n = 11) or moderate (n = 5) clinical improvement. The SaO2 improved from 90.7 +/- 5.3% to 96.0 +/- 4.1%. None of the patients had obstruction in the systemic venous pathway. In the 13 surviving patients with previous atriopulmonary anastomosis there was a drastic reduction in right atrial size. Four of 13 patients with atrial dysrhythmias converted to sinus rhythm. The right pulmonary vein compression as present in three patients resolved after conversion. Pleural effusions disappeared in four patients. CONCLUSIONS: Conversion to an extracardiac cavopulmonary connection may lead to clinical improvement in patients with atriopulmonary or lateral atrial tunnel Fontan connection associated with specific target conditions such as obstruction, pulmonary vein compression, right atrial enlargement, atrial dysrhythmia, or atrial thrombus. The conversion operation should not be unduly delayed to prevent irreversible deterioration of clinical status with chronic rhythm disturbances or protein-losing enteropathy. The benefit of the conversion operation is questionable in patients with poor clinical condition and protein-losing enteropathy.

Adolescent↗
PubMed1999

Resection of a right atrial metastatic melanoma with unknown origin of primary tumor.

Cardiac involvement of malignant melanoma is generally part of a widespread tumor dissemination, which is mostly multifocal. Hence the disease is usually not amenable to surgical intervention. We report successful resection of a large intracavitary melanoma to the right atrium, the primary origin of which was unknown. The right atrium was reconstructed with an autologous pericardial patch. At 12-month follow-up the patient remains asymptomatic.

Echocardiography, Transesophageal↗
PubMed1999

Resection of atherosclerotic aneurysm at origin of aberrant right subclavian artery.

In two patients (75- and 88-years-old), with the aid of cardiopulmonary bypass and using a transaortic approach, a rare calcified aneurysm at the origin of an aberrant right subclavian artery was closed with a prosthetic patch. The aneurysm was partially resected and the remainder closed over the patch. This technique obviates the need for hazardous clamping of the aorta around the base of the calcified aneurysm and allows secure closure of the origin of the aneurysm.

Aged↗
PubMed1998

Repair of complex unroofed coronary sinus by anastomosis of left to right superior vena cava.

An extracardiac repair technique is described for the anomalously connecting left superior vena cava in complex unroofed coronary sinus syndrome. In this technique, the left superior vena cava is divided distally and is anastomosed to the right superior vena cava in an end-to-side fashion; in addition, the intracardiac anomalies are corrected. The main advantage of this technique consists of avoidance of the various disadvantages of construction of a complex intraatrial baffle.

Anastomosis, Surgical↗
PubMed1998

Suspension of straddling tricuspid valve chordae into the appropriate ventricle.

In 2 children with an inlet ventricular septal defect and straddling chordae tendineae of the septal leaflet of the tricuspid valve to the posteromedial papillary muscle of the mitral valve and to an accessory papillary muscle in the left ventricle, the straddling chordae were excised with a wedge of posteromedial papillary muscle and with the top segment of the accessory papillary muscle, respectively. After patch closure of the ventricular septal defect, the papillary muscle segment with its group of chordae was anchored to the right ventricular septum with resulting competence of the tricuspid valve. In contrast to the traditional repair technique, the reported modification is applicable when the straddling chordae insert into a papillary muscle of the mitral valve. In addition, various disadvantages related to the construction of a complex baffle in the inappropriate ventricle are avoided.

Chordae Tendineae↗
PubMed1998

Modified repair of mixed anomalous pulmonary venous connection.

A modified repair technique is reported for mixed total or partial anomalous pulmonary venous connection with the right superior pulmonary vein connecting to the superior vena cava, the right inferior pulmonary vein to the right atrium or left atrium, and the left pulmonary veins to the coronary sinus. The superior vena cava is transected above the highest right superior pulmonary vein, its cephalad end is anastomosed to the right atrial appendage, and a pericardial baffle is constructed between the cardiac ostium of the superior vena cava, the ostium of the right inferior pulmonary vein, and the left atrium, including the coronary sinus, which is unroofed. The reported technique may be valuable to avoid pulmonary venous obstruction in complex mixed forms of total or partial anomalous pulmonary venous connection.

Anastomosis, Surgical↗
PubMed1998

Cephalad resuspension of aortic leaflet of the mitral valve in hypertrophic obstructive cardiomyopathy.

A young child with hypertrophic obstructive cardiomyopathy, who was reoperated on for recurrent left ventricular outflow tract obstruction, underwent myectomy with patch augmentation of the ventricular septum. Cephalad resuspension of the aortic leaflet of the mitral valve resulted in abolishment of residual obstruction occurring secondary to its systolic anterior motion.

Aortic Valve↗
PubMed1998

Pericardial patch augmentation of anterior tricuspid leaflet in Ebstein's anomaly.

Although refinement of tricuspid valvuloplasty and plication techniques has opened the way to a satisfactory outlook for the majority of patients with Ebstein's anomaly, paucity of tissue in the anterior tricuspid leaflet may preclude successful valve repair. In this particular circumstance, a modified Carpentier repair of the tricuspid valve with additional pericardial patch augmentation of the anterior leaflet may result in a well-functioning monocusp tricuspid valve.

Ebstein Anomaly↗