Journal of the American College of Cardiology, 2002
alous origin at an outside institution and has been lost to F/U. All pts s/p unroofing are asympt... more alous origin at an outside institution and has been lost to F/U. All pts s/p unroofing are asymptomatic with patent coronary flow by Doppler and normal/improved LV function at F/U; aortic insufficiency is mild in 1 (with bicuspid Ao valve), trivial in 1, and none in 3 pts. Conclusion: AOCA is frequently characterized by an intramural course which can be prospectively identified by TTE, The intramural form of AOCA can be reliably repaired by unroofing the intramural segment without bypass grafting, We speculate that eady TI'E identification and aggressive surgical intervention can be life-saving in pts with this rare anomaly.
A 10-YEAR-OLD GIRL WAS REFERRED FOR A cardiac evaluation due to a 12-month history of intermitten... more A 10-YEAR-OLD GIRL WAS REFERRED FOR A cardiac evaluation due to a 12-month history of intermittent palpitations, exercise induced dizziness, and chest-pain. Cardiac examination demonstrated a well-nourished African-American female with normal vital signs. The precordium was quiet and the apex beat was located in the fifth left intercostal space. There was a normal first heart sound but a widely split second heart sound and no audible murmurs. The chest was clear to auscultation and the liver was not enlarged. The electrocardiogram and 24 hour Holter monitor demonstrated normal sinus rhythm. An echocardiogram was performed to assess coronary arterial anatomy and demonstrated a small atrial septal defect of 5 mm diameter within the oval fossa, with no significant volume overload of the right heart. The aortic arch was left-sided, with no evidence of coarctation, but the coronary arterial origins could not be clearly visualized. In addition, there was a vascular structure arising from the inferior aspect of the aortic arch and coursing to the left, but its distal connection could not be visualized by echocardiography. Paracoronal maximal intensity projection of a three dimensional gadolinium magnetic resonance angiogram with sensitivity encoding technique (Figs 1 and 2b), along with sequential paracoronal tomographic magnetic resonance sections using the black blood double inversion turbo spin echo technique (Fig. 2a), were performed to evaluate this vascular anomaly. There was a double-barrelled aortic lumen, which was patent proximally and distally, representing persistence of the fifth aortic arch. The coronary arterial origins were normal, and the defect within the oval fossa was clearly visualized. An aorto-pulmonary collateral artery was also seen arising from the descending aorta.
Journal of the American College of Cardiology, 1987
Obstruction to pulmonary venous return may be associated with a number of congenital cardiovascul... more Obstruction to pulmonary venous return may be associated with a number of congenital cardiovascular abnormalities occurring both before and after surgery. Hemodynamic assessment by cardiac catheterization is often difficult. A noninvasive method for detection and quantitation of obstruction to systemic ventricular inflow would be clinically useful. Two-dimensionally directed pulsed and continuous wave Doppler echocardiography was performed before cardiac catheterization in 31 patients thought clinically to have possible obstruction to left ventricular inflow or pulmonary venous return. Primary diagnoses included transposition of the great arteries after the Mustard or Senning procedure in nine patients, total anomalous pulmonary venous connection in nine (in two after surgical repair), cor triatriatum in eight (in four after surgical repair), congenital mitral stenosis in four (in one after surgical repair) and mitral atresia in one. Severe obstruction was defined as a mean pressure gradient at catheterization of 2:16 mm Hg at any level of the pulmonary venous return or of the sys-In patients with congenital heart disease. obstruction to systemic ventricular inflow or to pulmonary venous return occurs relatively frequently both before and after surgery. Lesions that are particularly likely to be associated with such obstruction preoperatively are total anomalous pulmonary venous connection (1), cor triatriatum (2) and con-From the
BACKGROUND Patients with heterotaxy syndrome frequently have complex congenital cardiac and nonca... more BACKGROUND Patients with heterotaxy syndrome frequently have complex congenital cardiac and noncardiac malformations requiring detailed diagnostic evaluation by noninvasive as well as invasive imaging modalities for management planning. Recent advances in magnetic resonance imaging (MRI) techniques allow detailed delineation of cardiovascular anatomy and blood flow in young infants with rapid heart rates. The present study was undertaken to prospectively evaluate the role of MRI in the presurgical evaluation of patients with heterotaxy syndrome. METHODS AND RESULTS Between January 1 and December 31, 1992, 14 consecutive patients with heterotaxy syndrome and complex congenital heart disease were enrolled in a prospective protocol. After evaluation by echocardiography and cardiac catheterization, a tentative management plan was recorded. Subsequently, a MRI study was performed and surgical planning was reevaluated. MRI was found to be comparable to echocardiography in terms of length ...
Evaluation for patent ductus arteriosus by both Doppler examination and direct two-dimensional ec... more Evaluation for patent ductus arteriosus by both Doppler examination and direct two-dimensional echocardiographic visualization has been reported in infants and children. However, visualization of a patent ductus arteriosus in preterm infants with lung disease has been difficult. Using a recently developed 7.5 MHz mechanical scanner with interfaced two-dimensional directed pulsed Doppler ultrasonography, 36 examinations were performed from a suprasternal approach in 27 patients (age range 1 day to 3 months, mean 18 days; weight range 490 to 2,500 g, mean 1,260). Complete imaging for evaluation of patency of the ductus arteriosus was successful in 33 (92%) of 36 examinations, and imaging of the pulmonary end of the ductus arteriosus was successful in all. In 18 examinations, the ductus arteriosus was closed by both two-dimensional echocardiography and Doppler examination. In four cases the ductus arteriosus was widely patent by both two-dimensional echocardiography and Doppler examination. Eleven echocardiographic examinations revealed a narrowed ductus arteriosus, and of these, 10 (91%) showed Doppler findings of patent ductus arteriosus. It is concluded that combined two-dimensional/Doppler echocardiographic assessment allows confident detection of both a large unrestrictive and a small, stenotic patent ductus arteriosus in preterm infants with lung disease.
Journal of Cardiovascular Magnetic Resonance, 2007
Isolated anomalous origin of the right coronary artery from the main pulmonary artery (ARCAPA) is... more Isolated anomalous origin of the right coronary artery from the main pulmonary artery (ARCAPA) is a rare congenital cardiac malformation. We reviewed the current literature and found only 31 patients with ARCAPA. We report the first case that was diagnosed and followed on a noninvasive basis with cardiovascular magnetic resonance after surgical re-implantation. This report of a patient with ARCAPA showed resolving coronary artery sizes secondary to decreased pulmonary steal. Cardiovascular magnetic resonance is an accurate and reliable imaging modality that allows serial noninvasive follow up in patients with coronary artery anomalies.
Journal of the American College of Cardiology, 1995
For occlusion of patent ductus arteriosus IPDA) the currently used coil devices are not safe beca... more For occlusion of patent ductus arteriosus IPDA) the currently used coil devices are not safe because of lack of deployment precision and retrievability. In 1992 we developed a new intravascular occlusion technique using memory-shaped retrievable stainless steel coils (Duct-Occlud. Pfm. Cologne). A snap-in mechanism keeps the coil attached to the core wire before final detachment. The new occlusion technique, therefore enables precise placement of coils.
During the last few decades, significant strides have been made in the field of noninvasive imagi... more During the last few decades, significant strides have been made in the field of noninvasive imaging for the patient with congenital heart disease. Echocardiography and MRI continue to provide improved means of anatomic and functional assessment in children and adults with congenital heart lesions. This review reports some of the recent advances in tissue Doppler, strain rate, and integrated backscatter, and highlights exciting current and future potential developments in their application. We also discuss advances in MR in evaluation of cardiac anatomy and function in congenital heart disease.
Interrupted aortic arch is a rare congenital heart malformation occurring in approximately three ... more Interrupted aortic arch is a rare congenital heart malformation occurring in approximately three per 1 million births. Type B interrupted aortic arch (interruption between the second carotid artery and the ipsilateral subclavian artery) is the most common of three major types (A, B, and C). We report an extremely rare finding: a case of left-sided type B interrupted aortic arch with isolation of the right subclavian artery (origin from the right pulmonary artery).
Catheterization and Cardiovascular Interventions, 2000
Over the last two decades, surgical separation of thoracopagus conjoined twins has become increas... more Over the last two decades, surgical separation of thoracopagus conjoined twins has become increasingly feasible. Delineation of the degree of cardiac involvement is essential to establish the feasibility of surgical separation. We describe our experience of cardiac catheterization both in the diagnosis and management of nine sets of thoracopagus twins who presented to a single center over three decades and discuss its role in conjunction with other modern diagnostic modalities.
In patients with multiple blood transfusions, iron content was highest in liver, followed by the ... more In patients with multiple blood transfusions, iron content was highest in liver, followed by the spleen. Significant iron overload was detected in the myocardium of only one patient. Left ventricular systolic wall thickening was normal in patients receiving multiple blood transfusions. Two ...
Background and Purpose: Magnetic resonance imaging (MRI) can accurately and reproducibly measure ... more Background and Purpose: Magnetic resonance imaging (MRI) can accurately and reproducibly measure the volume of atherosclerotic plaque in human carotid arteries. Atherosclerotic plaques may either progress or regress over time, depending on individual risk factors and treatment regimens. This study was designed to determine if regression or progression of human carotid atherosclerosis in patients receiving statin therapy over 24 months can be detected by high-resolution MRI. Methods: In 11 subjects who had undergone unilateral carotid endarterectomy and were on statin therapy, volumes for total carotid artery, concentric wall (normal wall), eccentric wall (plaque), and lumen were quantified at 0, 16 and 24 months using a 1.5-T human imager equipped with 6-cm phased array coils. Results: The interobserver mean coefficient of variation (CV) was lowest for the lumen volume (3.1%) and highest for the plaque volume (9.8%). The interscan mean CV was lowest for the total artery volume (3.2%) and highest for the plaque volume (9.9%). As much as 26% regression and 35% progression were observed in individual subject's carotid artery eccentric wall (plaque) volumes over time. Mean eccentric wall volume increased 5% by 16 months and 8% by 24 months. Mean total wall volume increased slightly at both 16 and 24 months (+1.2% and +1.8%). Conclusions: High-resolution MRI provides a noninvasive reproducible method of tracking changes in carotid atherosclerosis. This pilot study detected changes in individual subjects at both 16 and 24 months. MRI tracking of changes in atherosclerotic plaques should prove useful in assessing vascular disease risk and monitoring the efficacy of interventions designed to induce regression or retard progression. D
Heart failure is an important cause of morbidity and mortality in individuals of all ages. The ma... more Heart failure is an important cause of morbidity and mortality in individuals of all ages. The many-faceted nature of the clinical heart failure syndrome has historically frustrated attempts to develop an overarching explanative theory. However, much useful information has been gained by basic and clinical investigation, even though a comprehensive understanding of heart failure has been elusive. Heart failure is a growing problem, in both adult and pediatric populations, for which standard medical therapy, as of 2010, can have positive effects, but these are usually limited and progressively diminish with time in most patients. If we want curative or near-curative therapy that will return patients to a normal state of health at a feasible cost, much better diagnostic and therapeutic technologies need to be developed. This review addresses the vexing group of heart failure etiologies that include cardiomyopathies and other ventricular dysfunctions of various types, for which current therapy is only modestly effective. Although there are many unique aspects to heart failure in patients with pediatric and congenital heart disease, many of the innovative approaches that are being developed for the care of adults with heart failure will be applicable to heart failure in childhood.
Scimitar syndrome is a rare anomaly involving abnormalities of the heart and lung which classical... more Scimitar syndrome is a rare anomaly involving abnormalities of the heart and lung which classically involves the right side. A rare case of left-sided scimitar syndrome is described in an asymptomatic child, with a review of the literature.
Journal of the American College of Cardiology, 2002
alous origin at an outside institution and has been lost to F/U. All pts s/p unroofing are asympt... more alous origin at an outside institution and has been lost to F/U. All pts s/p unroofing are asymptomatic with patent coronary flow by Doppler and normal/improved LV function at F/U; aortic insufficiency is mild in 1 (with bicuspid Ao valve), trivial in 1, and none in 3 pts. Conclusion: AOCA is frequently characterized by an intramural course which can be prospectively identified by TTE, The intramural form of AOCA can be reliably repaired by unroofing the intramural segment without bypass grafting, We speculate that eady TI'E identification and aggressive surgical intervention can be life-saving in pts with this rare anomaly.
A 10-YEAR-OLD GIRL WAS REFERRED FOR A cardiac evaluation due to a 12-month history of intermitten... more A 10-YEAR-OLD GIRL WAS REFERRED FOR A cardiac evaluation due to a 12-month history of intermittent palpitations, exercise induced dizziness, and chest-pain. Cardiac examination demonstrated a well-nourished African-American female with normal vital signs. The precordium was quiet and the apex beat was located in the fifth left intercostal space. There was a normal first heart sound but a widely split second heart sound and no audible murmurs. The chest was clear to auscultation and the liver was not enlarged. The electrocardiogram and 24 hour Holter monitor demonstrated normal sinus rhythm. An echocardiogram was performed to assess coronary arterial anatomy and demonstrated a small atrial septal defect of 5 mm diameter within the oval fossa, with no significant volume overload of the right heart. The aortic arch was left-sided, with no evidence of coarctation, but the coronary arterial origins could not be clearly visualized. In addition, there was a vascular structure arising from the inferior aspect of the aortic arch and coursing to the left, but its distal connection could not be visualized by echocardiography. Paracoronal maximal intensity projection of a three dimensional gadolinium magnetic resonance angiogram with sensitivity encoding technique (Figs 1 and 2b), along with sequential paracoronal tomographic magnetic resonance sections using the black blood double inversion turbo spin echo technique (Fig. 2a), were performed to evaluate this vascular anomaly. There was a double-barrelled aortic lumen, which was patent proximally and distally, representing persistence of the fifth aortic arch. The coronary arterial origins were normal, and the defect within the oval fossa was clearly visualized. An aorto-pulmonary collateral artery was also seen arising from the descending aorta.
Journal of the American College of Cardiology, 1987
Obstruction to pulmonary venous return may be associated with a number of congenital cardiovascul... more Obstruction to pulmonary venous return may be associated with a number of congenital cardiovascular abnormalities occurring both before and after surgery. Hemodynamic assessment by cardiac catheterization is often difficult. A noninvasive method for detection and quantitation of obstruction to systemic ventricular inflow would be clinically useful. Two-dimensionally directed pulsed and continuous wave Doppler echocardiography was performed before cardiac catheterization in 31 patients thought clinically to have possible obstruction to left ventricular inflow or pulmonary venous return. Primary diagnoses included transposition of the great arteries after the Mustard or Senning procedure in nine patients, total anomalous pulmonary venous connection in nine (in two after surgical repair), cor triatriatum in eight (in four after surgical repair), congenital mitral stenosis in four (in one after surgical repair) and mitral atresia in one. Severe obstruction was defined as a mean pressure gradient at catheterization of 2:16 mm Hg at any level of the pulmonary venous return or of the sys-In patients with congenital heart disease. obstruction to systemic ventricular inflow or to pulmonary venous return occurs relatively frequently both before and after surgery. Lesions that are particularly likely to be associated with such obstruction preoperatively are total anomalous pulmonary venous connection (1), cor triatriatum (2) and con-From the
BACKGROUND Patients with heterotaxy syndrome frequently have complex congenital cardiac and nonca... more BACKGROUND Patients with heterotaxy syndrome frequently have complex congenital cardiac and noncardiac malformations requiring detailed diagnostic evaluation by noninvasive as well as invasive imaging modalities for management planning. Recent advances in magnetic resonance imaging (MRI) techniques allow detailed delineation of cardiovascular anatomy and blood flow in young infants with rapid heart rates. The present study was undertaken to prospectively evaluate the role of MRI in the presurgical evaluation of patients with heterotaxy syndrome. METHODS AND RESULTS Between January 1 and December 31, 1992, 14 consecutive patients with heterotaxy syndrome and complex congenital heart disease were enrolled in a prospective protocol. After evaluation by echocardiography and cardiac catheterization, a tentative management plan was recorded. Subsequently, a MRI study was performed and surgical planning was reevaluated. MRI was found to be comparable to echocardiography in terms of length ...
Evaluation for patent ductus arteriosus by both Doppler examination and direct two-dimensional ec... more Evaluation for patent ductus arteriosus by both Doppler examination and direct two-dimensional echocardiographic visualization has been reported in infants and children. However, visualization of a patent ductus arteriosus in preterm infants with lung disease has been difficult. Using a recently developed 7.5 MHz mechanical scanner with interfaced two-dimensional directed pulsed Doppler ultrasonography, 36 examinations were performed from a suprasternal approach in 27 patients (age range 1 day to 3 months, mean 18 days; weight range 490 to 2,500 g, mean 1,260). Complete imaging for evaluation of patency of the ductus arteriosus was successful in 33 (92%) of 36 examinations, and imaging of the pulmonary end of the ductus arteriosus was successful in all. In 18 examinations, the ductus arteriosus was closed by both two-dimensional echocardiography and Doppler examination. In four cases the ductus arteriosus was widely patent by both two-dimensional echocardiography and Doppler examination. Eleven echocardiographic examinations revealed a narrowed ductus arteriosus, and of these, 10 (91%) showed Doppler findings of patent ductus arteriosus. It is concluded that combined two-dimensional/Doppler echocardiographic assessment allows confident detection of both a large unrestrictive and a small, stenotic patent ductus arteriosus in preterm infants with lung disease.
Journal of Cardiovascular Magnetic Resonance, 2007
Isolated anomalous origin of the right coronary artery from the main pulmonary artery (ARCAPA) is... more Isolated anomalous origin of the right coronary artery from the main pulmonary artery (ARCAPA) is a rare congenital cardiac malformation. We reviewed the current literature and found only 31 patients with ARCAPA. We report the first case that was diagnosed and followed on a noninvasive basis with cardiovascular magnetic resonance after surgical re-implantation. This report of a patient with ARCAPA showed resolving coronary artery sizes secondary to decreased pulmonary steal. Cardiovascular magnetic resonance is an accurate and reliable imaging modality that allows serial noninvasive follow up in patients with coronary artery anomalies.
Journal of the American College of Cardiology, 1995
For occlusion of patent ductus arteriosus IPDA) the currently used coil devices are not safe beca... more For occlusion of patent ductus arteriosus IPDA) the currently used coil devices are not safe because of lack of deployment precision and retrievability. In 1992 we developed a new intravascular occlusion technique using memory-shaped retrievable stainless steel coils (Duct-Occlud. Pfm. Cologne). A snap-in mechanism keeps the coil attached to the core wire before final detachment. The new occlusion technique, therefore enables precise placement of coils.
During the last few decades, significant strides have been made in the field of noninvasive imagi... more During the last few decades, significant strides have been made in the field of noninvasive imaging for the patient with congenital heart disease. Echocardiography and MRI continue to provide improved means of anatomic and functional assessment in children and adults with congenital heart lesions. This review reports some of the recent advances in tissue Doppler, strain rate, and integrated backscatter, and highlights exciting current and future potential developments in their application. We also discuss advances in MR in evaluation of cardiac anatomy and function in congenital heart disease.
Interrupted aortic arch is a rare congenital heart malformation occurring in approximately three ... more Interrupted aortic arch is a rare congenital heart malformation occurring in approximately three per 1 million births. Type B interrupted aortic arch (interruption between the second carotid artery and the ipsilateral subclavian artery) is the most common of three major types (A, B, and C). We report an extremely rare finding: a case of left-sided type B interrupted aortic arch with isolation of the right subclavian artery (origin from the right pulmonary artery).
Catheterization and Cardiovascular Interventions, 2000
Over the last two decades, surgical separation of thoracopagus conjoined twins has become increas... more Over the last two decades, surgical separation of thoracopagus conjoined twins has become increasingly feasible. Delineation of the degree of cardiac involvement is essential to establish the feasibility of surgical separation. We describe our experience of cardiac catheterization both in the diagnosis and management of nine sets of thoracopagus twins who presented to a single center over three decades and discuss its role in conjunction with other modern diagnostic modalities.
In patients with multiple blood transfusions, iron content was highest in liver, followed by the ... more In patients with multiple blood transfusions, iron content was highest in liver, followed by the spleen. Significant iron overload was detected in the myocardium of only one patient. Left ventricular systolic wall thickening was normal in patients receiving multiple blood transfusions. Two ...
Background and Purpose: Magnetic resonance imaging (MRI) can accurately and reproducibly measure ... more Background and Purpose: Magnetic resonance imaging (MRI) can accurately and reproducibly measure the volume of atherosclerotic plaque in human carotid arteries. Atherosclerotic plaques may either progress or regress over time, depending on individual risk factors and treatment regimens. This study was designed to determine if regression or progression of human carotid atherosclerosis in patients receiving statin therapy over 24 months can be detected by high-resolution MRI. Methods: In 11 subjects who had undergone unilateral carotid endarterectomy and were on statin therapy, volumes for total carotid artery, concentric wall (normal wall), eccentric wall (plaque), and lumen were quantified at 0, 16 and 24 months using a 1.5-T human imager equipped with 6-cm phased array coils. Results: The interobserver mean coefficient of variation (CV) was lowest for the lumen volume (3.1%) and highest for the plaque volume (9.8%). The interscan mean CV was lowest for the total artery volume (3.2%) and highest for the plaque volume (9.9%). As much as 26% regression and 35% progression were observed in individual subject's carotid artery eccentric wall (plaque) volumes over time. Mean eccentric wall volume increased 5% by 16 months and 8% by 24 months. Mean total wall volume increased slightly at both 16 and 24 months (+1.2% and +1.8%). Conclusions: High-resolution MRI provides a noninvasive reproducible method of tracking changes in carotid atherosclerosis. This pilot study detected changes in individual subjects at both 16 and 24 months. MRI tracking of changes in atherosclerotic plaques should prove useful in assessing vascular disease risk and monitoring the efficacy of interventions designed to induce regression or retard progression. D
Heart failure is an important cause of morbidity and mortality in individuals of all ages. The ma... more Heart failure is an important cause of morbidity and mortality in individuals of all ages. The many-faceted nature of the clinical heart failure syndrome has historically frustrated attempts to develop an overarching explanative theory. However, much useful information has been gained by basic and clinical investigation, even though a comprehensive understanding of heart failure has been elusive. Heart failure is a growing problem, in both adult and pediatric populations, for which standard medical therapy, as of 2010, can have positive effects, but these are usually limited and progressively diminish with time in most patients. If we want curative or near-curative therapy that will return patients to a normal state of health at a feasible cost, much better diagnostic and therapeutic technologies need to be developed. This review addresses the vexing group of heart failure etiologies that include cardiomyopathies and other ventricular dysfunctions of various types, for which current therapy is only modestly effective. Although there are many unique aspects to heart failure in patients with pediatric and congenital heart disease, many of the innovative approaches that are being developed for the care of adults with heart failure will be applicable to heart failure in childhood.
Scimitar syndrome is a rare anomaly involving abnormalities of the heart and lung which classical... more Scimitar syndrome is a rare anomaly involving abnormalities of the heart and lung which classically involves the right side. A rare case of left-sided scimitar syndrome is described in an asymptomatic child, with a review of the literature.
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Papers by Giles Vick