Obstructive sleep apnoea in craniofacial microsomia: analysis of 755 patients.

A retrospective cohort study was set up to analyse the prevalence and treatment of obstructive sleep apnoea (OSA) in relation to the severity of the deformity in patients with craniofacial microsomia (CFM). This study included a population of 755 patients with CFM from three craniofacial centres. Medical charts were reviewed for severity of the deformity, types of breathing difficulty, age at which breathing difficulty first presented, treatment for OSA, and treatment outcome. In total, 133 patients (17.6%) were diagnosed with OSA. Patients with Pruzansky IIB/III classification or bilateral craniofacial microsomia were significantly more often diagnosed with OSA than unilaterally affected patients of Pruzansky I/IIA classification. The initial treatment of OSA consisted of adenotonsillectomy, tracheotomy, or non-invasive positive pressure ventilation. Thirty-seven patients received more than one treatment (range 1-3). In this study, the prevalence of OSA in patients with CFM was higher than the prevalence in the healthy population described in the literature. Although several treatment modalities are available for the treatment of OSA in patients with CFM, treatment should be individualized and based on clinical symptoms, the severity of the deformity, and comorbidities.

Heart rate chaos in obstructive sleep apnea in children.

Obstructive sleep apnea syndrome (OSAS) in children is associated with a bradytachyarrhythmia during an obstructive event. Polysomnographic recordings were obtained from 15 children; 9 had OSAS (apnea/hypopnea index = 13.6 +/- 8.2/hr, mean +/- SD) and 6 normal controls. Heart rate variability was analyzed for the presence of chaotic dynamics. Using a 5-minute sliding window, chaos was detected using numerical titration technique. In both groups, REM had a higher chaotic intensity than NREM sleep (p < 0.05). Furthermore, chaos was significantly increased during periods with apneic events compared to stable breathing. These data indicate that sleep state and disordered breathing are important determinants of the autonomic control of heart rate chaos in children.

Abdominal aortic aneurysms and thoracic aortic atheromas.

BACKGROUND: Abdominal aortic aneurysm (AAA) is associated with atherosclerosis elsewhere. Thoracic aortic atheromas (ATHs) seen on transesophageal echocardiography (TEE) are an important cause of stroke and peripheral embolization. The purposes of this study were to determine whether an association exists between AAA and ATHs and to assess the importance of screening patients with ATHs for AAA. METHODS: For the retrospective analysis, 109 patients with AAA and 109 matched controls were compared for the prevalence of ATHs on TEE and for historical variables. For the prospective analysis, screening for AAA on ultrasonography was performed in 364 patients at the time of TEE. RESULTS: Results of the retrospective analysis showed that ATHs were present in 52% of patients with AAA and in 25% of controls (odds ratio [OR] = 3.3; P =.00003). There was a significantly higher prevalence of hypertension, myocardial infarction, heart failure, smoking, and carotid or peripheral arterial disease in patients with AAA. However, only ATHs were independently associated with AAA on multivariate analysis (P =.001). Results of the prospective analysis showed that screening at the time of TEE in 364 patients revealed AAA in 13.9% of those with ATHs and in 1.4% of those without ATHs (P <.0001; OR = 11.4). CONCLUSIONS: (1) There is a strong, highly significant association between abdominal aneurysm and thoracic atheromas. (2) Patients with AAA may be at high risk for stroke because of the concomitance of thoracic aortic atheromas. (3) The high prevalence of abdominal aneurysm in patients with thoracic atheromas suggests that screening for abdominal aneurysm should be carried out in all patients with thoracic atheromas identified by TEE.

Unusual echocardiographic views of bicuspid and tricuspid pulmonic valves.

The pulmonary artery is typically seen on transthoracic echocardiography in its longitudinal axis. Therefore, short axis views of the pulmonic valve leaflets are not generally obtained, and the distinction between tricuspid and bicuspid pulmonic valves is difficult or impossible. Bicuspid pulmonic valve is one cause of pulmonic stenosis, which is especially common in tetralogy of Fallot. Presented here are 2 patients in whom the orientation of the pulmonary artery was unusual, and the pulmonic valve was seen en face. The first patient had tetralogy of Fallot and a bicuspid pulmonic valve. The severe obstruction to right ventricular outflow was infundibular. The second patient had severe stenosis of a tricuspid pulmonic valve, which was treated with balloon valvuloplasty. These unusual views of the pulmonic valve leaflets were obtained because of anterior displacement of the pulmonary artery, and precise anatomic delineation of the problem in each case was possible with transthoracic echocardiography.

Respiratory flutter syndrome: an underrecognized cause of respiratory failure in neonates.

We report the clinical and respiratory data of three neonates with flutter of the diaphragm and intercostal muscles, presenting soon after birth with respiratory failure. The breathing pattern was dirhythmic with superimposed frequencies, one regular and slow (60/min) representing the underlying respiratory rate, the other fast (> 300/min) and limited to inspiration. Nasal continuous positive airway pressure immediately normalized the breathing pattern in one infant, and improved ventilation in the two others. Pharmacologic therapy with chlorpromazine terminated the respiratory flutter and permitted weaning of ventilatory support within a few hours. Coexistent dysphagia suggested a disorder of brainstem function, although the children were otherwise developmentally normal at 8, 10, and 26 mo old. Laryngomalacia and gastroesophageal reflux were also present. We propose that the occurrence of respiratory flutter, dysphagia, laryngomalacia, and gastroesophageal reflux in a neonate constitutes a distinct clinical entity, termed the "respiratory flutter syndrome." The diagnosis of three infants with this presentation during an 18-mo period suggests that this may be a more frequent cause of respiratory failure in newborns than previously recognized.

Pulmonary venous flow in large, uncomplicated atrial septal defect.

BACKGROUND: The pulmonary venous flow velocity pattern (PVFVP) in atrial septal defect (ASD) has not been previously studied in detail. Normally, PVFVP is primarily determined by the left heart performance. We hypothesized that the impact of left-sided heart dynamics on PVFVP is diminished in patients with ASD because of the presence of a left-to-right shunt into the low-resistance right side of the heart. METHODS AND RESULTS: Transesophageal echocardiography was performed in 19 adults and 3 children with a large, uncomplicated secundum ASD (maximum diameter 0.6 to 3.0 cm). All patients were in normal sinus rhythm with an average heart rate of 78 bpm in adults and 116 bpm in children. In 21 subjects the antegrade PVFVP lacked distinct systolic (S) and diastolic (D) waves. Instead, we observed a single continuous antegrade wave extending from the beginning of systole to the onset of atrial contraction. Furthermore, the amplitude of the atrial reversal (AR) wave was smaller than in historical controls. In 3 patients in whom ASD was surgically repaired, we observed an immediate return of distinct S and D waves postoperatively. This confirmed that PVFVP abnormality was indeed the result of the ASD. Also a large increase in the AR wave amplitude (46 + 15 cm/s) was noted postoperatively. CONCLUSIONS: This previously unrecognized PVFVP comprising a single continuous antegrade wave and a diminished AR wave sheds new light on the hemodynamics of ASDs. Its presence may also alert the echocardiographer to the possibility of an ASD when the septal defect cannot be visualized directly.

Diagnosis of a giant coronary aneurysm with multiple imaging modalities.

Echocardiography demonstrated an 8-cm mass adjacent to the right side of the heart in a 79-year-old man with a history of hypertension and a repaired abdominal aortic aneurysm. The results of Doppler echocardiography and magnetic resonance imaging suggested the diagnosis of an unusually large coronary artery aneurysm, and this was confirmed with coronary angiography. At surgery, the 8- to 10-cm coronary aneurysm was resected, and the patient made an uneventful recovery.

Surgical left atrial appendage ligation is frequently incomplete: a transesophageal echocardiograhic study.

OBJECTIVES: This study sought to determine the incidence of incomplete ligation of the left atrial appendage (LAA) during mitral valve surgery. BACKGROUND: Ligation of the LAA to prevent future thromboembolic events is commonly performed during mitral surgery. However, success in completely excluding the appendage from the circulation has never been systematically assessed. METHODS: Using transesophageal Doppler echocardiography, we studied 50 patients who underwent mitral valve surgery and ligation of the LAA. Thirty patients were studied immediately postoperative, and 20 patients were studied 6 days to 13 years after surgery. Incomplete ligation was detected by demonstrating a color jet traversing the separation between the left atrial body and appendage. RESULTS: Transesophageal echocardiography detected incomplete LAA ligation in 18 of 50 (36%) patients. The incidence of incomplete ligation was not significantly different between patients studied immediately postoperative and patients studied at various times after surgery. Type of mitral surgery (repair vs. replacement), operative approach (sternotomy vs. port access), left atrial size or degree of mitral regurgitation did not significantly correlate with the incidence of incomplete appendage ligation. However, the power to detect a significant difference in left atrial size was only 64%. Spontaneous echo contrast or thrombus was identified within appendages in 9 of 18 (50%) patients with incomplete ligation, while 4 of these 18 (22%) patients had thromboembolic events. CONCLUSIONS: Surgical LAA ligation is frequently incomplete. The similar incidence of incomplete ligation detected immediately postoperative and at various times thereafter suggest that this results from an intraoperative phenomenon rather than from gradual dehiscence of sutures over years. The incidence of incomplete left atrial ligation was unrelated to type of surgery, surgical approach, left atrial size or degree of mitral regurgitation. Residual communication between the incompletely ligated appendage and the left atrial body may produce a milieu of stagnant blood flow within the appendage and be a potential mechanism for embolic events.

Aortic valve replacement in patients with aortic stenosis and severe left ventricular dysfunction.

BACKGROUND: The outcome of aortic valve replacement for severe aortic stenosis is worse in patients with impaired left ventricular function. Such dysfunction in aortic stenosis may be reversible if caused by afterload mismatch, but not if it is caused by superimposed myocardial infarction. METHODS: From our echocardiography database, 55 patients with severe aortic stenosis (valve area < or =0.75 cm2) and ejection fractions of 30% or lower who subsequently underwent aortic valve replacement were included. The operative mortality and clinical follow-up were detailed. RESULTS: There were 10 perioperative deaths (operative mortality, 18%). Twenty (36%) of the 55 patients had a prior myocardial infarction. In the 35 patients without prior myocardial infarction, there was only 1 death (3%). In contrast, 9 of 20 patients with prior myocardial infarction died (mortality rate, 45%; P< or =.001). The factors significantly associated with perioperative death on univariate analysis (functional class, mean aortic gradient, and prior myocardial infarction) were entered into a model for stepwise logistic regression. This multivariate analysis showed that only prior myocardial infarction was independently associated with perioperative death (odds ratio, 14.9; 95% confidence interval, 2.4-92.1; P = .004). CONCLUSIONS: The risk of aortic valve replacement in patients with severe aortic stenosis and severely reduced left ventricular systolic function is extremely high if the patients have had a prior myocardial infarction. This information should be factored into the risk-benefit analysis that is done preoperatively for these patients, and it may preclude operation for some.

Late-onset central hypoventilation with hypothalamic dysfunction: a distinct clinical syndrome.

Idiopathic central hypoventilation has occasionally been reported in previously well children after infancy. The relationship between this late-onset central hypoventilation syndrome (LO-CHS) and congenital central hypoventilation syndrome (CCHS) has not been established. Both CCHS and LO-CHS have been associated with neural crest tumors, such as ganglioneuroblastoma and ganglioneuroma, and they generally occur in the presence of a histologically normal central nervous system. At least 10 case reports of idiopathic LO-CHS featured evidence of hypothalamic dysfunction (HD), including hyperphagia, hypersomnolence, thermal dysregulation, emotional lability, and endocrinopathies. We report on a case of LO-CHS/HD successfully treated by nasal intermittent positive pressure ventilation (NIPPV). Despite the commonalties with CCHS, we propose that LO-CHS/HD is a distinct clinical syndrome. In addition to the markedly different age at presentation, features of hypothalamic dysfunction are not seen in CCHS. Review of the literature was undertaken to further clarify the full spectrum of the disease.

Effects of intraaortic balloon counterpulsation on carotid artery blood flow.

BACKGROUND: The intraaortic balloon pump has been shown to improve cardiac output and diastolic coronary flow. Animal studies with balloon counterpulsation have shown variable effects on carotid and cerebral blood flow. We investigated the effects of counterpulsation with the intraaortic balloon pump on blood flow in the common carotid artery in human beings. METHODS AND RESULTS: We studied 14 patients who had an intraaortic balloon pump placed for clinical indications; 9 were hypotensive (4 in the setting of an acute myocardial infarction and 5 immediately after cardiac surgery). Five patients required counterpulsation after undergoing complicated coronary angioplasty. Common carotid artery blood flow was assessed with carotid Duplex scanning both with and without 1:1 balloon augmentation. We found no net increase in the total blood flow in the common carotid artery with intraaortic balloon counterpulsation. This result occurred despite a significant mean increase in both the peak flow velocity and flow velocity integral of the augmented diastolic flows by 160% and 78%, respectively. Total flow did not change because an early systolic reversal of blood flow was seen only with balloon augmentation, which negated the augmented diastolic flow. CONCLUSION: Despite a significant augmentation in diastolic blood flow with balloon counterpulsation, no increase occurred in total carotid artery blood flow. This appeared to be caused by improper timing of balloon deflation. This could result in no improvement in cerebral blood flow with intraaortic balloon pump counterpulsation in critically ill patients.

Sequential external counterpulsation increases cerebral and renal blood flow.

The purpose of this study was to evaluate the effect of sequential external counterpulsation (SECP) on cerebral and renal blood flow. The effect of SECP on carotid and renal artery blood flow was studied in 35 and 18 patients, respectively. With a portable unit, cuffs were applied to the calves and thighs, sequentially inflated with air at the onset of diastole, and deflated at the onset of systole. Carotid and renal artery Duplex studies were performed during intermittent SECP. Flow velocity and flow velocity integral were measured at baseline and during SECP. Diastolic augmentation of carotid and renal artery flow velocity was observed in all patients. The mean carotid flow velocity integral increased by 22% from 27.7 +/- 1.8 cm to 33.1 +/- 2.3 cm (P = 0.001). The mean renal artery flow velocity integral increased by 19% from 21 +/- 1 cm to 25 +/- 1 cm (P = 0.0001). With SECP, a new diastolic Doppler flow velocity wave was observed, with an average peak carotid diastolic flow velocity of 56 +/- 4 cm/sec and an average peak renal artery diastolic flow velocity of 40 +/- 2.5 cm/sec. This diastolic wave was 75% (carotid) and 68% (renal) as high as the systolic wave during SECP. In addition, with SECP the systolic wave increased by 6% and 8% in the carotid and renal artery, respectively (P = 0.02 and 0.006, respectively). In conclusion, SECP significantly increases carotid and renal blood flow. This noninvasive, harmless treatment may be useful to support patients with decreased cerebral and renal perfusion.

Correlation between plasma homocyst(e)ine and aortic atherosclerosis.

Plasma homocyst(e)ine [H(e)] levels correlate with the prevalence of arterial occlusive diseases. Recently, transesophageal echocardiography (TEE) has been used to evaluate patients with atherosclerotic plaques in the thoracic aorta. The purpose of this study was to determine whether H(e) levels correlate with the degree of atherosclerotic plaque in the thoracic aorta (ATH) as seen on TEE. Maximum plaque areas for three locations in the thoracic aorta (arch, proximal descending, and distal descending) were measured with TEE in 156 patients. Maximum plaque areas for these locations were added to yield an estimate of ATH. ATH and H(e) levels, and levels of folic acid, vitamin B12, and pyridoxal 5'-phosphate were measured in a double-blind manner. Univariate analysis demonstrated a significant correlation of H(e) with ATH (r = 0.3, p< 0.001). On multivariate analysis, H(e) was independently predictive of ATH (r for the model including H(e) was 0.63, p < 0.0001). Plasma H(e) levels are therefore significantly and independently correlated with the degree of atherosclerosis in the thoracic aorta.

Tortuosity of the descending thoracic aorta simulating dissection on transesophageal echocardiography.

In an 80-year-old patient with syncope, a markedly tortuous descending thoracic aorta produced images on transesophageal echocardiography which were suggestive of an intimal flap caused by dissection. A magnetic resonance aortogram clearly showed that the trans-esophageal echocardiogram was a false positive. In addition, multiplanar reconstructed images of the magnetic resonance aortogram through the tortuous descending thoracic aorta could reproduce images similar to that seen by transesophageal echocardiography. Because transesophageal echocardiography has become a popular imaging modality for the detection of aortic dissection, it is essential for echocardiographers to be aware of possible pitfalls which may create false positive findings.

Incomplete occlusion of left ventricular aneurysms after endoventricular aneurysmorrhaphy: diagnosis by echocardiography and ventriculography.

Surgical treatment of left ventricular aneurysms have recently focused on maintaining normal left ventricular geometry by using a circular patch repair to exclude the aneurysmal cavity (endoaneurysmorrhaphy). We describe two patients who underwent this procedure and were subsequently found by echocardiography and angiography to have a residual communication between the left ventricular cavity and the aneurysm which contained thrombus. This finding may have implications regarding the optimal hemodynamic result of the surgery and the risk of thromboembolism.

Echocardiographic evaluation of the coronary sinus.

The purpose of this study was to compare transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) in the evaluation of the coronary sinus and its blood flow. Forty patients were studied by TTE and TEE. The distal coronary sinus and its right atrial communication could be identified in 21 of 40 by TTE, and in all patients by TEE. Coronary sinus diameter measurement at the right atrial communication was possible by TTE in 16 of 40, and in all patients by TEE (maximal diameter 6 to 14 mm, mean 9 +/- 2). Flow velocity measurement by pulsed Doppler was possible in 25 of 40 patients (63%) by TEE, and in none by TTE. The flow velocity pattern was similar to central vein flow velocity, with systolic and diastolic antegrade waves, and a small retrograde end diastolic wave. The coronary sinus cross-sectional area was measured in 5 patients by intravascular ultrasound. It varied in size and shape during the cardiac cycle, reaching a maximum (0.3 to 1.5 cm2) at end diastole, and decreasing by 40% to 70% at end systole. TEE is superior to TTE in the evaluation of the coronary sinus and its blood flow velocity. However, because of the variability in cross-sectional area size and shape, measurement of coronary sinus blood flow may be inaccurate.

High risk for vascular events in patients with protruding aortic atheromas: a prospective study.

OBJECTIVES: The purpose of this study was to prospectively evaluate the risk of vascular events in patients with protruding aortic atheromas. BACKGROUND: Protruding atheromas of the thoracic aorta have been shown to be associated with embolic disease in previous retrospective studies. METHODS: During a 1-year period, 521 patients had transesophageal echocardiography. Of these, 42 patients had protruding atheromas and no other source of emboli. They were followed up for up to 2 years (mean follow-up 14 months) and compared with a control group without atheromas, matched for age, gender and hypertension. RESULTS: Of 42 patients with atheromas, 14 (33%) had 19 vascular events during follow-up (5 brain, 2 eye, 4 kidney, 1 bowel, 7 lower extremity). Of 42 control patients, 3 (7%) had vascular events (2 brain, 1 eye). Univariate analysis identified only protruding atheromas as significantly correlating with events (p = 0.003). There was no positive correlation of events with age, gender, hypertension, smoking, family history, atrial fibrillation, valve replacement, antithrombotic drug use, diabetes or coronary disease. Multivariate analysis showed that only protruding atheromas independently predicted events (p = 0.005, odds ratio 4.3, 95% confidence interval 1.2 to 15.0). Nine patients died in the atheroma group versus six in the control group, but this was not statistically significant (p = 0.39). CONCLUSIONS: Protruding atheromas seen on transesophageal echocardiography predict future vascular events.