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R Kaulitz

Publications and source records attributed to R Kaulitz.

At least 19 recordsLinked to original sources
PubMed2006

[Echocardiographic diagnosis of the aorto-pulmonary window].

UNLABELLED: PURPOSE/MATERIALS AND METHODS: The aorto-pulmonary window is a rare congenital cardiac malformation consisting of a connection between the ascending aorta and the main pulmonary artery. Based on 5 consecutive patients who were treated in our tertiary referral centre between 7/2000 and 11/2005, we report the echocardiographic features as well the limitations of this diagnostic method in the assessment of this rare malformation. RESULTS: Dilation of the left atrium and ventricle due to the large left-to-right-shunt was the characteristic echocardiographic feature in all patients with aorto-pulmonary window. In all patients we were able to visualise the window by 2D-echocardiography. Pulsed- and continuous-wave Doppler showed diastolic negative flow in the descending aorta and in systemic arteries due to left-to-right shunting in diastole via the aorto-pulmonary window. All 5 patients had significant associated cardiovascular malformations. They were accurately diagnosed by echocardiography. Coronary anomalies could be excluded noninvasively in all patients. CONCLUSION: Echocardiography is the method of choice for diagnosis of aortopulmonary window. Detection of aortopulmonary window, however, requires a systematic and careful investigation of all cardiovascular anatomic details, since this anomaly is frequently associated with substantial additional cardiac anomalies, which would by themselves be sufficient to explain the haemodynamic abnormalities of the patient. Cardiac catheterisation is required only in cases with inadequate information about coronary artery anatomy or in older patients with pulmonary hypertension, to determine the patient's suitability for surgical correction.

Aorta, Thoracic↗
PubMed2005

Recanalisation of bilateral superior vena cava after total cavopulmonary connection. Interventional occlusion with the Amplatzer VSD Occluder.

UNLABELLED: Formation of systemic to pulmonary venous or systemic venous left atrial collaterals frequently occurs in patients after Glenn or Fontan-type operations. Embolization with detachable metal coils is the therapy of choice for the closure of small vessels. These devices however are not appropriate for the occlusion of large collaterals, e. g. recanalized bilateral caval veins. We report two patients who presented late after Fontan-type operations with a gradual decrease in oxygen saturation due to recanalisation of bilateral caval veins. Interventional closure of these large veins was carried out successfully with the use of 8 mm Amplatzer muscular VSD Occluders, resulting in an increase of arterial oxygen saturations. CONCLUSION: The closure of recanalized bilateral superior caval veins after Fontan procedures is possible without technical problems by means of the Amplatzer muscular VSD Occluder. In order to avoid future formation of venous collaterals via the azygos or hemiazgos system, the occluder should be placed in the vena cava below the orifice of the azygos/ hemiazygos vein.

Adult↗
PubMed2005

[Cervical origin of the subclavian artery: echocardiographic diagnosis in patients with monosomy 22q11].

OBJECTIVE: To assess the sensitivity and specificity of echocardiography in the detection of anomalies of the subclavian artery (cervical origin, aberrant origin from the descending aorta or isolation) in patients with monosomy 22q11. METHOD: From 1/1998 until 03/2002 we examined 57 patients with conotruncal cardiac malformations. 30 patients had pulmonary atresia and ventricular septal defect (53 %), 13 interrupted aortic arch (23 %), 9 tetralogy of Fallot (16 %) and 5 common truncus arteriosus (9 %). Echocardiographic examination included identification of the laterality of the aortic arch as well as examination of the origin of the brachiocephalic vessels. The results were compared with angiographic and intraoperative findings. Median age at echocardiographic investigation was 74 days (range 1 d - 33.4 yrs.). RESULTS: Laterality of the aortic arch was assessed correctly in all patients. 20/57 patients had a right-sided aortic arch (35 %). Echocardiography detected all anomalies of the subclavian artery (14/57 patients, 25 %, sensitivity 100 %) and excluded these anomalies correctly in 43 patients (specificity 100 %). Exact classification of the anomaly of the subclavian artery was possible in 6/6 patients with a cervical origin of the artery and in 7/8 patients with aberrant origin from the descending aorta. Monosomy 22q11 was diagnosed in 21 patients (37 %). CONCLUSION: Echocardiography achieves a high sensitivity in the detection of anomalies of the subclavian artery. The diagnosis of cervical origin of the artery, in particular, can be easily established. As this anomaly appears to be a specific marker for monosomy 22q11, echocardiography facilitates reliable identification of these patients in clinical practice.

Adolescent↗
PubMed2005

Current treatment and prognosis in children with functionally univentricular hearts.

For more than 30 years Fontan-type procedures have been performed for surgical treatment in patients with functionally univentricular hearts. Advances in proper patient selection, staging of the Fontan palliation, and modifications of the surgical technique resulted in continuous improvement of the surgical results during the past two decades. However, increasing experience with medium term and long term follow-up of the Fontan circulation has drawn attention to a number of sequelae and chronic complications affecting a significant number of patients.

Fontan Procedure↗
PubMed2004

Excision of the coronary orifices in arterial switch operation: "O" like obstructive and "U" like unobstructive?

BACKGROUND: Stenoses of the neo-pulmonary artery (NPA) may complicate follow-up of the arterial switch operation (ASO). It is unknown whether the type of patch covering the coronary excision defects ("O"- or "U"-shaped) might influence this complication. METHODS: Echocardiographically and invasively measured NPA pressure-gradients were evaluated retrospectively in 95 children after ASO. Median follow-up was 5.8 years. Defects had been covered with pericardial patches: O/O and U/U (left/right) 34 x each, and 27 x in mixed combinations. The frequency of NPA stenoses requiring re-interventions was registered. RESULTS: Median of the peak instantaneous echocardiographic pressure gradient was 23 mmHg (interquartile range, IQR: 16 - 49, n = 34) in O/O, and 19 mmHg (IQR: 13 - 23, n = 34) in U/U; p < 0.034, t-test. Invasively measured gradients were 49 mmHg (IQR: 17 - 65, n = 12) in O/O, and 12 mmHg (IQR: 7-21, n = 28) in U/U; p < 0.001. One child per O/O- and U/U-group underwent balloon angioplasty of the neo-pulmonary root. Five children of the O/O-group had to undergo repeat surgery, whereas only one child in the U/U-group required repeat surgery ( p < 0.34). CONCLUSIONS: The preferential type of covering the NPA coronary excision sites in ASO should be U-shaped.

Anastomosis, Surgical↗
PubMed2003

[Asymptomatic cor triatriatum in a 2 1/4 year old patient].

We report on an asymptomatic 2 1/4 year old girl. Echocardiography revealed cor triatriatum with a membrane separating the left atrium. Transesophageal echocardiography demonstrated 3 large perforations of the intraatrial membrane. Using Doppler sonography, no pressure gradients across these orifices were identified. Obstruction of the intraatrial membrane and pulmonary hypertension were excluded by cardiac catheterization. We therefore decided against surgical resection of the membrane and to follow the patient noninvasively by echocardiography. Follow-up over 18 months revealed no development of an obstruction across the intraatrial membrane. Our case shows that immediate surgical correction is not necessary in all patients with cor triatriatum. Conservative management of these patients requires full evaluation of the hemodynamics and careful follow-up examinations by echocardiography.

Child, Preschool↗
PubMed2003

[Echocardiographic diagnosis of the aorto-left ventricular tunnel].

AIM/METHOD: The aorto-left ventricular tunnel (ALVT) is a rare congenital cardiac malformation with clinical findings of severe aortic insufficiency. We report the echocardiographic differentiation of different anatomical variants of ALVT in two infants. RESULTS: Echocardiography in both patients demonstrated severe enlargement of the ascending aorta and the left ventricle. In the first patient the ALVT originated from the ascending aorta above the right coronary sinus and entered the left ventricle just below the aortic valve. In the second patient the ALVT originated above the left coronary sinus and took a lateral course to the left ventricle. Colour-Doppler-sonography in both patients confirmed a systolic-diastolic flow across the tunnel. Many patients have associated cardiac defects. Exact determination of the morphology of the aortic valve and coronary arteries is mandatory for surgical repair. Postoperative follow-up studies focus on the function of the aortic valve and the left ventricle. CONCLUSION: Differentiation of different anatomical variants of ALVT is possible trough echocardiography. Cardiac catheterization is required only in cases with inadequate information about coronary artery anatomy.

Aortic Valve↗
PubMed2002

[Tetralogy of Fallot with right pulmonary artery origin from the ascending aorta: noninvasive diagnosis and surgical correction in a 7-week-old infant].

Anomalous origin of one pulmonary artery from the ascending aorta is a rare anomaly which is almost always associated with hypertension in the contralateral pulmonary artery originating from the right ventricle. We report echocardiographic evaluation of an infant with tetralogy of Fallot and aortic origin of the right pulmonary artery. Since all relevant information regarding anatomy and hemodynamics could be obtained by echocardiography, cardiac catheterization and angiography were unnecessary. Surgical correction including transatrial patch closure of the ventricular septal defect, resection of the infundibular stenosis and direct reimplantation of the right pulmonary artery was performed successfully at the age of 7 weeks. The postoperative course was uneventful and persistent pulmonary hypertension was excluded by echocardiography. Our case shows that complete echocardiographic evaluation is possible in infants with this complex cardiac malformation. We recommend reserving cardiac catheterization for those patients with echocardiographic evidence of significant elevation of pulmonary vascular resistance. In order to avoid irreversible pulmonary vascular changes early surgical correction within the first 3 months of life should be performed in all children with aortic origin of a pulmonary artery including those with associated tetralogy of Fallot.

Aorta↗
PubMed2002

[Transcatheter closure of atrial septal defects with the CardioSEAL occluder].

Data on mid- and long-term follow-up for the recent devices for closure of secundum-type atrial septal defects are limited. The purpose of our retrospective study was to report the effectiveness of transcatheter closure in patients with various morphological types of atrial septal defect other than centrally located defects within the oval fossa using the CardioSEAL and CardioSEAL-Starflex occluder. A total of 91 patients (age 1.5-71 years, median 6 years) underwent transcatheter closure. On the transesophageal echocardiogram, defect size varied from 6 to 18 mm with an estimated stretched diameter of 11 to 24 mm, median 15 mm; the ratio of the stretched diameter to septal length ranged from 0.28 to 0.68. Mean follow-up was 28.7 +/- 11,9 months (range 3-46 months). Isolated secundum-type defects were present in 59 patients (65%), multiple septal defects including patients with perforated atrial septal aneurysms and defects with deficient atrial rim in 32 patients (35%). Occlusion rate using device diameters from 23 to 40 mm increased from 66% (60/91 patients) immediately after implantation to 86% (48/56 patients) 24 months after implantation. Patients with isolated secundum-type defects presented with a significantly higher primary closure rate (45/59 patients, 76%) compared to patients with various defect morphology. Closure rate did not depend on the type of implanted device modification. No thrombus formation, sustained atrial arrhythmia or infective endocarditis occurred. Serial transthoracic echocardiographic findings revealed protrusion of one left-sided arm onto the right atrial aspect in 5 patients; malposition of one right-sided superior arm of the device was observed in 7 patients. Fluoroscopy showed single fatigue fracture in 7 patients (7.7%) within the first 6 months after implantation. These results demonstrate that transcatheter closure with the double umbrella device was effective and safe on medium-term follow-up and could be extended to atrial septal defects of various morphology.

Adolescent↗
PubMed2001

Atrial reentrant tachycardia after surgery for congenital heart disease: endocardial mapping and radiofrequency catheter ablation using a novel, noncontact mapping system.

BACKGROUND: The purpose of the present study was to determine the role of a novel, noncontact mapping system for assessing a variety of atrial reentrant tachycardias (ART) in patients after the surgical correction of congenital heart disease. METHODS AND RESULTS: In 14 patients, an electrophysiological study using the Ensite 3000 system was performed to assess ARTs resistant to medical treatment. Sixteen different forms of ART were inducible in the 14 patients studied. The reentrant circuit of all ARTs could be characterized and localized with respect to anatomic landmarks such as atriotomy scars, intraatrial patches/baffles, and cardiac structures. In 15 of the 16 ARTs (in 13 of the 14 patients), a target area of the reentrant circuit for radiofrequency current application (ie, an area of conduction between 2 anatomical obstacles such as surgical barriers and cardiac structures of electrical isolation) could be localized within the systemic venous atrium. Nine patients exhibited macroreentry, and 4 showed microreentry. In 12 patients, ART could be terminated by creating linear radiofrequency current lesions (75 degrees C, 180 to 390 s). Completeness of linear lesions after radiofrequency current delivery was proven by analyzing color-coded isopotential maps of atrial activation while applying atrial pacing techniques. The mean duration of the procedures was 286 minutes (range, 130 to 435 minutes); fluoroscopy time ranged from 7 to 33.8 minutes (mean, 17.4 minutes). CONCLUSIONS: In patients with ART after the surgical correction of congenital heart disease, the use of the noncontact mapping system allows for characterization of the tachycardia and guidance for effective radiofrequency current delivery.

Adolescent↗
PubMed2001

Transcatheter closure of various types of defects within the oval fossa using the double umbrella device (CardioSEAL)--feasibility and echocardiographic follow-up.

Data on long-term follow-up for closure of so-called secundum type" atrial septal defects within the oval fossa using recently developed devices are limited, and results focused on presence of residual shunting. The purpose of our study was to report the experience from a single center establishing the effectiveness of transcatheter closure in patients with various types of defect other than those located centrally within the oval fossa. A total of 72 patients was included in this study. On transesophageal echocardiography, the size of the defects varied from 6 to 18 mm, with estimation of the stretched diameter from 11 to 21 mm. The ratio of stretched diameter to the extent of the residual septum ranged from 0.28 to 0.54. Mean follow-up was 30.5+7.4 months, with a range from 13 to 42 months. The rate of closure using devices with diameters from 28 to 40 mm increased from 80% immediately after implantation to 93% in the 57 patients examined 24 months after implantation. For further analysis, we compared the 44 patients with a solitary, centrally located, defect to 28 having morphological variations, including superiorly located defects with deficient superior and aortic rims, multifenestrated and aneurysmal defects, or isolated additional defects. There was no incidence of formation of thrombus, sustained atrial arrhythmia, or infective endocarditis. Residual shunting was not influenced by location or morphology of the defects, but increased with size, stretched diameter, and the ratio of pulmonary to systemic flows. Serial transthoracic echocardiographic findings revealed malposition of one right-sided superior arm of the device in 8 patients, while protrusion of one left-sided arm onto the right atrial aspect was observed in 3 patients. Fluoroscopy showed fatigue fracture of a single arm in 7 patients (9.7%) within the first 6 months after implantation. These results demonstrate that transcatheter closure with the non self-centering double umbrella device was effective and safe on medium-term follow-up, and could be extended to defects within the oval fossa having various morphologies. Residual shunting resolved with time, and was not related to either morphology or the position of the device.

Adolescent↗
PubMed2001

Primary repair of tetralogy of fallot in infancy--the effect on growth of the pulmonary arteries and the risk for late reinterventions.

We sought to analyse the long-term follow-up after primary repair of tetralogy of Fallot in infancy in the first year of life, paying particular attention to growth of the pulmonary arteries and the need for reintervention. We performed a combined retro- and prospective echocardiographic study, including measurements of the pulmonary valve and right and left pulmonary arteries, indexed to the square root of body surface area, in 62 patients prior to primary repair, 18 to 24 months after this event, and at the most recent follow-up, with a mean of 80.4+/-24 months. Of these, 38 patients, with an age at operation of 5.0+/-3.4 months, had presented preoperatively with hypoxic spells or increasing cyanosis. The remaining 24 patients had been asymptomatic, with adequate flow of blood to the lungs. Their age at elective operation was 7.4+/-3.0 months. A transannular patch was needed in 37 patients (63%). There were 3 early postoperative deaths (4.8%). Cross-sectional echocardiography revealed a significantly smaller diameter for the pulmonary valve in patients who had been symptomatic preoperatively compared to the asymptomatic patients (1.09 versus 1.3 cm/BSA0.5; p = 0.019). The diameters of the right and left pulmonary arteries did not differ significantly between the groups. Examination of echocardiographic data obtained 18 to 24 months postoperatively in 43 patients revealed a significant increase in the diameter of the pulmonary arteries; 0.83+/-0.17 cm/BSA0.5 versus 1.1+/-0.26 cm/BSA0.5 for the diameter of the right pulmonary artery, 0.85+/-0.2 cm/BSA0.5 versus 1.0+/-0.25 cm/BSA0.5 for the left pulmonary artery. On comparison between individuals, 18.6% and 25.6% of the patients, respectively, did not show any change in the diameters of their right and left pulmonary arteries, whereas the increase in diameter reached or exceeded the measurements in normal controls in 55.8% and 46.5% of the patients, respectively. On recent follow-up, with a range from 56 to 147 months, no further increase in the indexed diameters of the pulmonaries could be documented in 21 of 33 patients. Of these, 9.1% and 18.2%, respectively, presented with small right and left pulmonary arteries compared with measurements obtained in normal controls. Moderate pulmonary incompetence was found on colour flow mapping in one-third. Of 56 longterm survivors, 8 (14%) had required reinterventions, which were surgical in 6 and achieved by transcatheter techniques in the other 2 patients. Thus, primary correction of tetralogy of Fallot in infancy, with restoration of normal pressures and flows, resulted in sustained increase in the diameters of the right and left pulmonary arteries. It allowed for early normal development of the proximal pulmonary arterial system in most patients regardless of their age and symptomatic status at operation. Patients with persistent subnormal diameter of the pulmonary arteries did not present with significantly elevated right ventricular pressure. Early one-stage repair of tetralogy of Fallot in infancy was associated with a low rate of reinterventions.

Age Factors↗
PubMed2000

Transcatheter retrieval and repositioning of an Amplatzer device embolized into the left atrium.

A transcatheter technique is described for stabilization and retrieval of an embolized Amplatzer device, which was inadvertently deployed in the left atrium of a 2-year-old girl with hemodynamically significant atrial septal defect within the fossa ovalis. Since surgery was not available for immediate device retrieval, transcatheter means were chosen to prevent embolization of the device into the mitral valve. After stabilizing the device by creating a guidewire circuit through the wire mesh of the right atrial disk, the Amplatzer device was repositioned through the interatrial defect by snaring the microscrew. No residual shunting and perfect device position was recorded during Doppler echocardiography directly after the procedure as well as 1, 2, and 120 days after the intervention. Cathet. Cardiovasc. Intervent. 51:297-300, 2000.

Cardiac Catheterization↗
PubMed2000

[Antegrade flow in the aorta ascendens despite aortic atresia: 2 case reports with retrograde coronary perfusion through coronary fistulas and sinusoids].

In aortic atresia, coronary perfusion normally occurs through retrograde blood flow in the ascending aorta. We report on two patients with antegrade flow in the ascending aorta despite aortic atresia. In one patient with hypoplastic left heart syndrome (aortic atresia, severe mitral stenosis), an intact interatrial septum/premature closure of the foramen ovale was found. While no other way of left atrial or ventricular decompression was found, echocardiography, angiography and the post-mortem examination showed left ventricular to coronary sinusoids as the sole pathway for systemic oxygenation. In a second patient with complex congenital heart disease, including aortic atresia, antegrade flow in the ascending aorta was through a left coronary fistula with shunt flow originating from the pulmonary trunk. This report describes systemic perfusion depending on retrograde coronary flow due to coronary-cameral (sinusoids) and coronary arterio-venous fistulas leading to the phenomenon of antegrade blood flow in the ascending aorta despite aortic atresia.

Aorta↗
PubMed2000

[Atrioventricular nodal reentry tachycardia in children: curative treatment by high frequency catheter ablation].

BACKGROUND: Atrioventricular nodal reentrant tachycardia (AVNRT) is one of the most common forms of supraventricular tachycardia in the pediatric population. PATIENTS AND METHODS: 41 children with a mean age of 9.6 (3.7-16) years with recurrent atrioventricular nodal reentrant tachycardia (AVNRT) refractory to medical treatment (n = 38) and recurrent syncope (n = 3) underwent electrophysiologic (EP) study. In all patients dual AV-nodal physiology could be demonstrated during EP study and typical form of AVNRT (mean heart rate 220/min) could be induced by programmed atrial stimulation. A steerable 7 F ablation catheter was placed at the inferoparaseptal region of the tricuspid valve annulus close to the orifice of the coronary sinus with the intention to record a late fractionated local atrial electrogram during sinus rhythm. Starting at this point radiofrequency current (500 kHz) with a target temperature of 70 degrees C was delivered with the intention to ablate the slow pathway. If a slowly accelerated junctional rhythm (< 120/min) occurred during energy discharge, programmed atrial stimulation was repeated. Otherwise radiofrequency current was delivered step by step up to a septal position next to the tricuspid valve annulus. Slow pathway ablation was defined as lack of evidence of dual AV nodal pathways during repeated atrial stimulation. Slow pathway modulation was defined as maximal one atrial echoimpulse after ablation. RESULTS: The number of energy applications ranged from 1-19 (median 6). In 35/41 patients slow pathway ablation could be achieved; in six patients the slow pathway was modulated. In none of the patients permanent high grade AV block was observed. During follow-up (mean 4.1 years) two patients had a recurrent episode of AVNRT after slow pathway modulation. All other patients are still free of AVNRT without medical treatment. CONCLUSION: Selective radiofrequency current ablation/modulation of the slow pathway is a safe and curative treatment of AVNRT in young patients.

Adolescent↗
PubMed2000

A novel method for transcatheter closure of atrial septal defect within an aneurysm of the fossa ovalis: double sheath technique.

An aneurysm of the fossa ovalis is frequently associated with an atrial septal defect. Intervention with transcatheter closure of such defects may be difficult since it bears the risk of inadvertent deployment of the device with both umbrellas in the right or left atrium. A novel technique for the closure of such multiperforated aneurysms of the fossa ovalis, which allows central positioning and controlled deployment of the device, is reported.

Aortic Dissection↗
PubMed1999

Transcatheter closure of atrial septal defect with a new flexible, self-centering device (the STARFlex Occluder).

The purpose of this study was to evaluate the short-term safety and efficacy of the STARFlex Occluder for transcatheter closure of atrial septal defects with difficult anatomy. Transcatheter closure of the defect was attempted and successfully performed in 5 patients; the "stretched" defect diameter ranged from 12 to 24 mm and the implanted devices ranged from 23 to 33 mm.

Cardiac Catheterization↗