Acute and follow-up intravascular ultrasound findings after balloon dilation of coarctation of the aorta.

BACKGROUND: The study objective was to examine the vascular wall changes caused by balloon dilation of coarctation of the aorta (CoA) acutely and at short-term follow-up using intravascular ultrasound imaging. Intravascular ultrasound has been valuable in assessing the vessel wall changes in coronary and peripheral arteries after balloon dilation, often with more detail than angiography. METHODS AND RESULTS: Intravascular ultrasound imaging, using 4.8F, 20-MHz or 6.2F, 12.5-MHz catheters on either Diasonics or HP scanners, was performed in 17 patients during balloon angioplasty for native (n = 12) and recurrent (n = 5) CoAs. Nine patients were also studied at the time of follow-up cardiac catheterization 28.1 +/- 18.0 months after angioplasty. Immediately after dilation, the mean pressure gradient across the CoA decreased from 42.9 +/- 16.4 to 9.0 +/- 5.4 mm Hg (P < .001) and the mean diameter of the coarcted segment increased from 4.4 +/- 1.9 to 7.9 +/- 2.4 mm (P < .001). An intimal tear or flap was noted by ultrasound in 12 of the 12 native CoAs and 4 of the 5 recoarctations. In contrast, only 6 of the native CoAs and 2 of the recoarctations had an intimal flap or dissection detected by angiography. At follow-up, the residual pressure gradient did not significantly change from that measured immediately after dilation, but the CoA diameter increased from 7.8 +/- 1.5 to 9.9 +/- 2.3 mm (P < .01). No aneurysms were detected. Four of the 9 patients showed ultrasonic and angiographic evidence of healing and remodeling with diminution in size or disappearance of the intimal tears. CONCLUSIONS: There is a high incidence of intimal tears and dissections immediately after balloon angioplasty for native and recurrent CoAs. Intravascular ultrasound is more sensitive than angiography in detecting the vascular wall changes. Even significant intimal tears are not necessarily associated with aneurysm formation, and many decrease in size or disappear at short-term follow-up.

Therapeutic cardiac catheterization in children.

Cardiac catheterization, once the mainstay of diagnosis in children with congenital heart disease, has become a therapeutic modality for many conditions. Balloon dilatation can now open stenotic valves and vessels, coils and umbrellas can now close unwanted communications, and emboli can be withdrawn without surgical intervention.

Elucidation of the natural history of ventricular septal defects by serial Doppler color flow mapping studies.

Two-dimensional echocardiography has provided information to aid in the diagnosis and management of infants with ventricular septal defect, but its inability to resolve very small ventricular septal defects and problems with defining ventricular septal defect orifice size (because of overlying muscle or tricuspid tissue) have made it unsuitable as a standard for defining the natural history of ventricular septal defect. In this study, 114 serial two-dimensional Doppler color flow mapping studies were performed to define ventricular septal defect anatomy, location and color flow diameter as an indicator of shunt size in 66 patients (over a 40 month period). Twenty-five patients first studied at 6 months of age (mean age at most recent study 15.9 months) had congestive heart failure and 41 (mean age 45 months) did not. In the congestive heart failure group, there were 24 perimembranous and 1 muscular ventricular septal defect and aneurysm formation was present in 17. Mean (+/- SD) color flow diameter was 8.2 +/- 1.9 mm and color flow diameter/aortic root diameter ratio was 0.63. In the 30 patients who underwent cardiac catheterization, color flow diameter bore a close relation to angiographic diameter (r = 0.96) and pulmonary/systemic flow ratio (Qp/Qs) (r = 0.88). In the patients with congestive heart failure, 4 of the 25 ventricular septal defects, all with aneurysm present or positioned adjacent to the tricuspid valve, became smaller but none closed. Of the 41 patients without congestive heart failure, 21 had a perimembranous defect (15 with aneurysm), 18 had a muscular ventricular septal defect and 2 had a supracristal ventricular septal defect.(ABSTRACT TRUNCATED AT 250 WORDS)

Color Doppler flow mapping in patients with coarctation of the aorta: new observations and improved evaluation with color flow diameter and proximal acceleration as predictors of severity.

We performed color Doppler flow mapping in 15 patients, 1 week to 17 years old (mean 42 months), with coarctation of the aorta that was confirmed subsequently by angiography and/or surgery. Twelve patients had native coarctation and three had mild recoarctation after surgical repair. Color Doppler flow maps were analyzed with a digital analysis package and a Sony computer system. The diameter in the region of coarctation from the color Doppler flow map (mean = 2.0 +/- 0.8 mm [SD]) correlated well with the coarctation diameter measured at angiography (mean = 1.8 +/- 0.8 mm; r = .83, SEE 0.43 mm) in the 10 patients with native coarctation undergoing angiography, but the coarctation diameter measured by two-dimensional echocardiography (3.9 +/- 1.5 mm) was poorly predictive of the angiographic severity (r = .23). Additionally, spatial acceleration was seen in all patients proximal to the coarctation site, with an aliased and accelerating stream narrowing progressively as it proceeded toward the coarctation site, a pattern that is not seen in healthy subjects. Computer analysis of the color Doppler images provided pseudo three-dimensional and digital velocity maps for blue, red, and green (turbulent) flow velocities to allow an enhanced appreciation of the accelerating stream, easily separating this from normal descending aortic aliasing patterns. The narrowing of the acceleration area in the proximal descending aorta (distal/proximal acceleration zone ratio) was also predictive of the angiographic severity of coarctation (r = .83). The distribution of low-level turbulence seen proximally paralleled the distribution of the proximal accelerating stream.(ABSTRACT TRUNCATED AT 250 WORDS)

Noninvasive Doppler color flow mapping for detection of anomalous origin of the left coronary artery from the pulmonary artery and for evaluation of surgical repair.

Anomalous origin of the left coronary artery from the pulmonary artery is a rare but important cause of congestive heart failure in infancy and of sudden death at all ages. Diagnosis is often missed when based solely on physical examination and noninvasive methods. A 4 year old patient is presented in whom mitral regurgitation was noted by a referring physician and an anomalous left coronary artery was found by Doppler color flow mapping upon referral and verified at cardiac catheterization. Doppler color flow mapping was also used intraoperatively using a gas-sterilized transducer to further clarify the hemodynamics and assess the surgical result. After creation of an intrapulmonary artery tunnel from the ostium of the left coronary artery to the aorta, anterograde coronary artery flow and absence of a residual left to right pulmonary artery shunt were verified during surgery by Doppler flow mapping. Postoperatively, residual mitral regurgitation and patency of the left coronary artery graft have been followed up serially by Doppler flow mapping. Therefore, Doppler color flow mapping is useful in the diagnosis and intraoperative and postoperative management of this important and potentially life-threatening abnormality.

Real-time Doppler color flow mapping for detection of patent ductus arteriosus.

In this study, ultrasound Doppler color flow mapping systems were utilized to examine flow in the pulmonary artery in 31 premature and term infants (aged 4 hours to 9 months) with patent ductus arteriosus accompanying respiratory distress syndrome, as an isolated lesion, or with patent ductus in association with other cyanotic or acyanotic congenital heart disorders. The flow mapping patterns were compared with those of a control population of 15 infants who did not have patent ductus arteriosus. In unconstricted ductus arteriosus, the flow from the aorta into the pulmonary artery was detected in late systole and early diastole and was distributed along the superior leftward lateral wall of the main pulmonary artery from the origin of the left pulmonary artery back in a proximal direction toward the pulmonary valve. In constricted patent ductus arteriosus, or especially in a ductus in association with cyanotic heart disease, the position of the ductal shunt in the pulmonary artery was more variable, often directed centrally or medially. Waveform spectral Doppler sampling could be performed in specific positions guided by the Doppler flow map to verify the phasic characteristics of the ductal shunt on spectral and audio outputs. Shunts through a very small patent ductus arteriosus were routinely detected in this group of infants, and right to left ductal shunts could also be verified by the Doppler flow mapping technique. This study suggests substantial promise for real-time two-dimensional Doppler echocardiographic flow mapping for evaluation of patent ductus arteriosus in infants.

Renal function in children after large dose contrast medium angiocardiography.

Serum creatinine and routine urinalysis were obtained and glomerular filtration rate estimated in 56 consecutive and hemodynamically stable children with congenital heart disease undergoing cardiac catheterization and angiocardiography, a day prior to, 1 and 7 days following the procedure. None had a history of renal disease. The patients were divided into 2 groups: Group I, 32 patients who received less than 3 ml/Kg of Renagrafin 60 and Group II, 24 patients who received greater than 3 ml/Kg. Despite a slight rise of creatinine on the first day, there were no statistically significant changes in serum creatinine or glomerular filtration rate for either group following the procedure. Occult blood was present in the urine of 2 in Group I and 1 in Group II prior to cardiac catheterization; this resolved in all cases after the procedure. Three patients in Group I and 1 in Group II developed transient trace proteinuria following the angiographic procedure. It is concluded that in children with congenital heart disease and normal kidney function, substantial doses of standard contrast medium administered during angiocardiography do not produce any apparent impairment of renal function.

Right bundle branch block in complete transposition.

Two of 26 infants with complete transposition presented with complete right bundle branch block in the first year of life, and 10 showed incomplete right bundle branch block. In no instance was the right ventricular conduction anomaly related to surgery or cardiac catheterization and it was not present at birth. The two cases with complete right bundle branch block had prolonged and severe hypoxemia and markedly dilated right ventricles. One had depressed right ventricular function and died after a Mustard procedure from low output syndrome. Whereas incomplete right bundle branch block may reflect right ventricular hypertrophy, it is suggested that complete right bundle branch block in complete transposition may signify right ventricular dysfunction and possibly irreversible changes of the right ventricular myocardium. It may be a sequel of long-term hypoxemia and pressure overload. Once observed, it is perhaps an indication for an alternative surgical approach other than an intra-atrial repair procedure.

Right pulmonary artery--left atrial communication. A case report and literature review.

A 22-month-old girl with Down's syndrome with a direct communication between the right pulmonary artery and left atrium leading to systemic desaturation is presented. She also had an atrial septal defect of the secondum type, which spontaneously closed. She underwent successful ligation of the abnormal communication. The literature is reviewed and, considering the high incidence of cerebral and systemic emboli, early surgical intervention is recommended.

Prostaglandin E1 responsive ductus at 11 months of age.

An 11-month-old boy with congestive heart failure and an intermittently closing patent ductus arteriosus (PDA) is presented. During cardiac catheterization, the ductus proved responsive to prostaglandin E1. Permanent closure of the PDA could not be attained with indomethacin. The infant underwent surgical ligation of the PDA.

Total anomalous pulmonary venous connection. Report of ten cases and review of the literature.

Ten cases of total anomalous pulmonary venous connection, all presenting at or below the age of eight weeks, are presented: three below the diaphragm, two to the coronary sinus, one to the right atrium, three to the left vertical vein, and one to the right superior vena cava. Six patients had other cardiac anomalies, and surgical mortality represented 75%. A review of the subject is presented.

Electrocardiographic recognition of sinus node dysfunction in children and young adults.

Twenty-four children and young adults with electrocardiographic evidence of sinus node dysfunction (SND) are described. The patients, whose ages ranged from three days to 25 years, were divided into three groups based on the type of SND. Ten patients had persistent and inappropriate sinus bradycardia (group I): twelve patients had episodes of prolonged sinus arrest (group II); and seven patients had repeated episodes of Mobitz type I or II sinoatrial exit block (group III). Eighteeen patients had significant heart disease and in 11 of these the SND followed corrective cardiac surgery. Seven were operations for atrial septal defect and transposition repair. Seven patients with SND were symptomatic, having either syncopal (3) or near syncopal episodes (4). All three with syncope have received permanent demand pacemakers. These data show that sinotrial exit block occurs in many young patients with SND (29% in this series). Its recognition, however, requires careful electrocardiographic analysis in order to differentiate sinus bradycardia and sinus arrhythmia. Although SND most commonly occurs in children with congenital or acquired heart disease, particularly following corrective cardiac surgery, it may occur in the absence of other cardiac abnormalities.