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Inadvertent disruption of tricuspid valve apposition by a pacing catheter in a neonate is described. Severe cyanosis due to atrial level shunting resulted, similar to the pathophysiologic state seen in Ebstein's anomaly.
Persistent cyanosis after successful balloon valvuloplasty for neonatal critical pulmonary valve stenosis is often related to poor right ventricular compliance and right-to-left shunting at the atrial level. A successful catheter closure of an atrial septal defect was performed with a dramatic increase in systemic oxygen saturation alleviating the need for a surgical systemic-to-pulmonary artery shunt.
Eighteen months after sustaining a stab wound to the left upper chest, a 59-year-old man presented with cyanosis and extertional dyspnea. Arterial desaturation due to a central 22 per cent right-to-left shunt was present. A selective pulmonary arteriogram demonstrated a fistula between the main pulmonary artery and the left atrium. At operation the fistula was closed. A laceration of the pulmonic valve and healed pericarditis were present. Marked symptomatic improvement followed the operation, but a murmur of pulmonic valvular regurgitation persisted. The fistula and laceration of the pulmonic valve were probably traumatic in origin.
Congenital diaphragmatic herniation into the pericardial cavity is extremely rare. We report a case associated with an ostium secundum atrial septal defect and normal pulmonary vascular resistance, which resulted in severe episodic cyanosis.
The expected level of systemic arterial saturation may not be present after bidirectional superior cavopulmonary anastomosis in children with complex congenital cardiac anomalies. We present a case of persistent severe cyanosis in a patient with azygos continuation of the inferior vena cava after bidirectional superior cavopulmonary anastomosis (Kawashima procedure) due to an intrahepatic venovenous malformation. Subsequent transcatheter deployment of two Rashkind double umbrella devices into the malformation reduced the shunt and markedly improved systemic arterial saturation.
A case of central cyanosis occurring after coronary artery bypass graft surgery is described. This was due to the development of a right ventricular infarction which facilitated shunting from right to left through a previously undiagnosed patent foramen ovale or small atrial septal defect.
Tachypnea and cyanosis in the newborn are frequently encountered problems in the nursery. The incidence of respiratory distress ranges from 2.9% to 7.6%, and 4.3% of newborns may require supplemental oxygen therapy. In this article, the pathophysiology,approach to the diagnosis, and management of clinical conditions are discussed. The physiologic mechanisms involved in the pathogenesis of these problems and the clinical assessment and early stabilization of an infant before transport to a tertiary care center, if necessary, are also discussed.
We describe a rare case of cyanosis following the Fontan operation secondary to right-to-left shunting through a pulmonary vein connected to both the left atrium and the hepatic veins. This is a hazardous anomaly in a Fontan candidate and should be considered in those with unexpected postoperative desaturation.
To elucidate differences in myocardial blood flow and metabolism between cyanotic and normal hearts, a model of chronic cyanosis was created in five adult mongrel dogs by anastomosing the inferior vena cava to the left atrium. After 6 to 9 months, myocardial blood flow, the ratio of subendocardial to subepicardial flow, oxygen consumption, oxygen extraction ratio, and lactate consumption in these cyanotic dogs and five control dogs were determined under baseline conditions and during pharmacologic stress with isoproterenol (0.2 micrograms/kg/min). Radioactive microspheres were used to determine left and right ventricular blood flow rates, and arterial and coronary sinus differences in oxygen and lactate levels were measured. At baseline and during stress, oxygen consumption and oxygen extraction ratios were identical in control and cyanotic hearts. Total myocardial blood flow was increased with stress and did not differ between cyanotic and control hearts. Left ventricular muscle from cyanotic hearts did exhibit lower endocardial/epicardial blood flow ratios than those of control hearts at rest, and the relative subendocardial flow decreased further with stress. During isoproterenol infusion, myocardial lactate production, indicative of anaerobic metabolism, was evident in two of five cyanotic animals and none of the control dogs. The relative subendocardial ischemia and its further aggravation by stress in cyanotic hearts may contribute to the pathophysiologic basis of myocardial dysfunction in cyanotic heart disease.
Congenital bladder diverticula are an uncommon cause of urinary retention in children. We report on a male infant with bilateral congenital bladder diverticula that compressed the posterior urethra causing urinary retention and also compressed the venous return from the lower extremities producing cyanosis.
The infant with cyanosis is a distressing medical presentation. The emergency room physician must be familiar with the diverse causes that may be underlying the event. An understanding of the pathophysiology along with a thorough history and physical examination will guide the physician toward appropriate intervention and management.
Cardiac rupture is a frequent cause of death following blunt trauma. Most of these patients die at the scene with only a few surviving to make it to the hospital. With improvements in prehospital care and rapid regional transport, more of these patients may arrive at the hospital with signs of life. Classically, these patients arrive with the constellation of symptoms that characterize cardiac tamponade (hypotension, distended neck veins, and distant heart sounds). The differential appearance of upper body cyanosis frequently accompanies these injuries. Prompt recognition and expeditious surgical treatment may increase the number of survivors of this catastrophic injury. Presented here is an illustrative case report and review of the literature.
A case is reported of a 37 year old man who was involved in an accidental shell blast. He was admitted with black tattooing of his face, forearms, hands and legs. Repair of the severe ocular lesions and the surgical debridement of his burns required general anaesthesia. This lasted for 6 h; throughout, haemodynamic and respiratory parameters remained stable. The Pao2 was 130 mmHg. During recovery, cyanosis was noted despite a Pao2 of 65 mmHg with 92% Sao2, a normal chest X-ray and stable normal haemodynamic parameters. This paradoxical situation suggested a toxic haemoglobinopathy. Biochemical investigations revealed severe methaemoglobinaemia (40%) due to percutaneous absorption of dinitrobenzene (DNB), a constituent of the 1917 bomb. The amount of DNB absorbed had been unknowingly reduced by the surgical brushing of the burned skin. The classical treatment of methaemoglobinaemia, associated with two plasmaphereses to remove the toxic substance, were successful. Normal arterial blood gases associated with chocolate brown coloured arterial blood should make one suspect methaemoglobinaemia.
The authors report here an unusual case of an ascending aorta aneurysm diagnosed in the event of a check-up for a dyspnea with cyanosis. The right atrium compression by the aneurysm initiated a right-to-left shunt through a patent foramen ovale.
Cyanosis as a result of right-to-left shunting across a ventricular septal defect is commonly encountered in patients with congenital heart disease when systolic pressure in the right ventricle exceeds that in the left ventricle. Reported is the case of a child who remained cyanosed after surgical correction of pulmonary atresia despite right ventricular systolic pressure being lower than left ventricular pressure. Colour-flow Doppler showed a residual ventricular septal defect, with right-to-left shunting in diastole alone.
Flutamide, an anti-androgen used in prostate cancer therapy, is also a derivative of aniline. Mild, usually asymptomatic, methaemoglobinaemia has been reported. We report a patient receiving flutamide therapy who developed cyanosis, dyspnoea and anaemia, initially attributed to marked methaemoglobinaemia by the CO-Oximeter method. An unsuccessful trial of methylene blue therapy led to the finding of marked sulphaemoglobinaemia. Sulphaemoglobinaemia has not previously been reported with flutamide use. Recognition of this association is important, given the refractoriness of sulphaemoglobinaemia to methylene blue therapy.
A 12-year-old girl with a solitary pulmonary arterio-venous fistula (P.A.V.F.) affecting the R.L.L. is reported. The cyanosis was noted since birth and increased with age. The diagnosis was confirmed by right heart catheterization and pulmonary arteriography. Preoperatively, her hematocrit was 73%, paO2 36 mm Hg and no bruit was heard. The treatment consisted of a right lower lobectomy. One year postoperatively, the patient has normal activities, is asymptomatic and has no evidence of residual disease. A short review of the literature on the subject of P.A.V.F. is included and indicates the relative rarity of surgical treatment of this disease in childhood.
An 18-year-old woman without previously documented medical history delivered a healthy 32-week-old preterm infant. Severe bleeding occurred during week 1 postpartum secondary to ruptured vaginal condylomas. The consequent anemia was accompanied by complaints of exercise intolerance that resolved with blood transfusion. Six weeks later, the patient was brought to the emergency department of the regional medical center, where she was found to be unresponsive, with a left-sided hemiparesis and with hypoxemia that failed to correct with assisted ventilation and 100% oxygen. A cardiology consultation was obtained, which identified the patient as having central cyanosis and heretofore unappreciated marked clubbing of fingers and toes. This suggested a diagnosis of right-to-left shunting, likely at the cardiac level, together with presumptive embolic stroke, which was documented by magnetic resonance imaging and magnetic resonance angiography as a left pontine infarct. Transthoracic and transesophageal echocardiography identified anomalous drainage of the inferior vena cava into the left atrium, which was confirmed by a three-dimensional computed tomographic angiogram. Corrective surgery in the form of atrial septostomy and redirection of the inferior vena cava to the right atrium was performed. The patient was subsequently discharged and is in stable condition 3 months later without effort intolerance and with normal arterial oxygen saturation on room air.