Stein Ji

Inhaled nitric oxide in patients with critical pulmonary perfusion after fontan-type procedures and bidirectional glenn anastomosis

OBJECTIVE The aim of this study was to evaluate the effects of inhaled nitric oxide in patients with critical pulmonary perfusion after Fontan-type procedures and bidirectional Glenn anastomosis. METHODS Inhaled nitric oxide (mean 4.1 +/- 0.7 ppm, 1.5 to 10 ppm) was administered in 13 patients (mean age 5.6 +/- 1.6 years, 1.5 to 17 years) with critical pulmonary perfusion (central venous pressure > 20 mm Hg or transpulmonary pressure gradient > 10 mm Hg) in the early postoperative period after total cavopulmonary connection (n = 9) or after bidirectional Glenn anastomosis (n = 4). RESULTS In patients after total cavopulmonary connection inhaled nitric oxide therapy decreased central venous pressure by 15.3% +/- 1.4% (p = 0.0001) and transpulmonary pressure gradient by 42% +/- 8% (p = 0.0008) and increased mean systemic arterial and left atrial pressures by 12% +/- 3.6% (p = 0.011) and 28% +/- 8% (p = 0.007), respectively. Arterial and venous oxygen saturations improved by 8.2% +/- 1% (p = 0.005) and 14% +/- 4.3% (p = 0.03), respectively. In patients after bidirectional Glenn anastomosis inhaled nitric oxide therapy resulted in a decrease of central venous pressure by 22% +/- 1% and of the transpulmonary pressure gradient by 55% +/- 6% and improved arterial and venous oxygen saturations by 37% +/- 29% and 11% +/- 3%, respectively. Mean systemic arterial and left atrial pressures remained nearly unchanged. No toxic side effect was observed in any patient. CONCLUSION Inhaled nitric oxide may play an important role in the management of transient critical pulmonary perfusion caused by reactive elevated pulmonary vascular resistance in the early postoperative period after Fontan-type operations and bidirectional Glenn anastomosis.

Catheter-directed thrombolysis with recombinant tissue plasminogen activator for acute pulmonary embolism after fontan operation.

Abstract. We report the case of a 3-year-old girl who presented with near-lethal pulmonary thrombembolism 3 weeks after an uneventful Fontan operation. Complete occlusion of the left lower lobe pulmonary artery had occurred together with a cerebral infarction. Recombinant tissue plasminogen activator (rt-Pa) was used for thrombolysis because of its short half-life and its clot-selective properties. To further minimize the systemic effects of rt-PA, local catheter-directed lysis was performed. A prolonged course of low-dose rt-PA therapy achieved complete lysis without side effects.

Atypical presentation of Kawasaki disease in an infant

SummaryA 7-month-old male infant with clinical symptoms of severe toxic shock syndrome died on day 9 of illness. At autopsy, demonstration of coronary vasculitis together with thrombosis of the left coronary artery revealed the true diagnosis of atypical Kawasaki disease. The marked similarity in many clinical features makes the distinction between these two diseases difficult when atypical clinical presentation of Kawasaki disease is present.

Transcatheter coil occlusion of residual interatrial communications after Fontan procedure

Objective To assess the use of detachable coils as an alternative method to occlude interatrial communications after Fontan operations. Design Descriptive clinical study of selected patients after Fontan operation with interatrial communications inappropriate for transcatheter umbrella occlusion. Setting Tertiary paediatric cardiac referral centre. Patients Seven patients after Fontan operation with residual interatrial communications of various types producing a right to left shunt. Interventions Transcatheter placement of detachable coils with a diameter of 3 or 5 mm within the interatrial communication. Results A total of 14 coils were successfully placed within persistent patent fenestrations of the interatrial baffle, residual leaks at the suture line between the patch material and the right atrial wall, and unusual venous interatrial communications. The mean (SD) aortic oxygen saturation increased from 88 (1.1)% (range, 86–89%) to 92 (1.3)% (range, 89–93%; p < 0.001) and the mean (SD) right atrial pressure rose from 9.7 (2) mm Hg (range, 6–11) to 10.6 (2.4) mm Hg (range, 6–13; p < 0.05) after coil implantation. In five patients, complete obliteration of the interatrial shunt was shown by angiography after coil implantation. At a mean (SD) follow up of 10 (4) months (range, 3–15) a residual interatrial shunt was detected by Doppler colour echocardiography in only one patient, and oxygen saturations ranged from 90% to 95% (mean, 92%). There were no late coil embolisations, thromboembolic events, or haemolysis in any patient. Conclusions Detachable coils can be used successfully to occlude residual interatrial communications after the Fontan procedure. In selected cases, in whom intended transcatheter umbrella occlusion of residual interatrial leaks is not possible, the use of detachable coils might offer a safe alternative method to eliminate interatrial right to left shunting after the Fontan procedure.

Dissection of the Descending Aorta After Balloon Angioplasty of Native Coarctation

Abstract. A 12-year-old girl underwent successful balloon angioplasty for a waist-like native coarctation. The balloon size/coarctation diameter ratio was 3.3. Postdilatation angiography showed a small aneurysm at the coarctation site. On frequent review the patient remained symptom-free and normotensive. Recatheterization was performed 14 months after balloon aortoplasty, when angiography revealed a massive aortic dissection extending from the origin of the left subclavian artery to both iliac arteries. She underwent partial replacement of the thoracic aorta. Balloon angioplasty of a narrow waist-like native coarctation may lead to extensive wall dissection and should be considered critically.

Extralobar sequestration with unusual arterial supply and venous drainage.

We present a case of extralobar pulmonary sequestration between the left lower lobe and diaphragm with an unusual arterial blood supply and venous drainage. Angiography revealed a large systemic artery arising from the left subclavian artery. The venous return paralleled this anomalous artery and drained into the left subclavian vein. This case illustrates the wide anatomic variability of such complex bronchovascular anomalies. Careful preoperative evaluation of both the arterial supply and venous drainage is important to avoid intraoperative complications. Angiography provides clear definition of these abnormal vascular structures, which is essential for appropriate therapeutic management.

Scimitar syndrome with absence of the right pulmonary artery: A case with volume-induced, reversible, left-sided pulmonary hypertension

SummaryAn infant with scimitar syndrome, absent right pulmonary artery, and systemic blood supply to the right lung presented in severe cardiac failure. Cardiac catheterization revealed suprasystemic pressure of the left pulmonary artery and a high pulmonary vascular resistance. Right-sided pneumonectomy abolished cardiac failure and normalized both pulmonary artery pressure and resistance. Pure volume load affecting one lung—as in this case through absence of the right pulmonary artery plus additional left-to-right shunt from a systemic collateral—can lead to pulmonary hypertension. Early operative intervention can reverse this process and prevent pulmonary vascular disease.

Continuous arteriovenous haemofiltration in children with postoperative cardiac failure.

Six children with refractory heart failure were treated by continuous arteriovenous haemofiltration. The cause of the failure was postoperative fluid overload or low cardiac output with anuria or oliguria. This produced a mean (2 SD) negative fluid balance of 1.4 (0.6) ml/kg/h and reduced mean (2 SD) body weight from 4.7 (2.2) to 4.2 (2.3) kg over a period of 57.5 (31.1) hours. Central venous pressure fell significantly from 13.7 (3.1) to 7.7 (0.7) mm Hg while the mean (2 SD) arterial pressure increased significantly from 44.6 (5.5) to 52.6 (5.1) mm Hg. In three infants urine production resumed when normal blood volume had been achieved. The other three infants needed further haemofiltration because of prolonged renal failure. All but one was weaned from artificial ventilation and catecholamine treatment. No adverse haemodynamic effects were noted. One child need operation for a femoral artery thrombosis after 12 days of continuous arteriovenous haemofiltration.

Mitral atresia with premature closure of the oval foramen, right-sided levoatriocardinal vein and thrombus formation in the left atrium.

We describe a 4-week-old baby with mitral atresia, hypoplastic left ventricle, ventricular septal defect, preductal coarctation and premature closure of the oval foramen whose only outlet from the left atrium was a stenosed right-sided levoatriocardinal vein and who in addition developed left atrial thrombi. Cross-sectional echocardiography was extremely helpful in establishing the diagnosis.

Interventioneller Verschluss von interatrialen Verbindungen nach modifizierter Fontanoperation

After modified Fontan operations various communications between the systemic and pulmonary venous returns may cause persistent or increasing postoperative cyanosis. Interventional closure of these right-to-left shunts may be necessary to eliminate hypoxemia and to reduce the risk of paradoxical embolic complications. Patients and methods Eighteen patients with a mean age of 5.6±4.1 (2.5–17.5) years underwent interventional closure of a right-to-left shunt 17.4±15.8 (3–60) months after a modified Fontan operation. After test balloon occlusion fenestrations were closed in 13 patients using an Amplatzer Septal occluder (n=7), a Rashkind PDA occluder (n=3), a CardioSeal umbrella (n=1) and detachable coils (n=2). Residual leaks at the suture lines between the interatrial patch and the right atrial wall were closed using detachable coils and a Rashkind PDA occluder in 2 and 1 patients, respectively. In 3 patients intracardiac venous collateral channels were closed by means of detachable coils. Results The mean aortic oxygen saturation increased from 85±4.5 (70–89)% to 91.4±2.8 (83–95)% (p<0.001) breathing room air and the mean tunnel pressure rose from 10.7±1.8 (6–14) mmHg to 12.1±2.4 (6–16) mmHg (p<0.001). Calculated Qs decreased from 5.15±2.1 (2.1–11.3) l/min/m2 to 3.6±1.0 (1.8–5.6) l/min/m2 (p<0.001). Mixed venous saturation (66.4±7.4% vs 65±7%) and mean systemic arterial pressure (73±8mmHg vs 73±9mmHg) remained unchanged. In one patient an additional leak of the tunnel could not be closed because of an increase to more than 18mmHg of the mean pressure in the lateral tunnel during balloon test occlusion. In 2 patients residual leaks after umbrella and coil occlusion of a fenestration and an additional venous collateral channel were closed by means of coils after 16 and 21 months, respectively. At a follow-up of 42±23 (7–99) months, mean oxygen saturation measured by pulse oxymetry was 93±2 (90–97)%. In 2 patients color-coded Doppler echocardiography revealed a minimal residual right-to-left shunt. In 2 patients contrast echocardiography demonstrated the additional presence of intrapulmonary fistulas. All patients remained free from device migration, thrombembolic events and hemolysis. Conclusion After modified Fontan operations various right-to-left shunts between the systemic and pulmonary venous returns can be successfully closed using umbrella devices or coils to eliminate cyanosis and to reduce the risk of paradoxical embolism. Nach einer modifizierten Fontanoperation können Kommunikationen unterschiedlicher Genese und Morphologie zwischen system- und pulmonalvenösem Stromgebiet eine persistierende oder postoperativ zunehmende Zyanose mit dem erhöhten Risiko einer paradoxen Embolie verursachen und damit ein operatives oder interventionelles Vorgehen erforderlich machen. Methode Bei 18 Patienten im mittlerem Alter von 5,6±4,1 (2,5–17,5) Jahren wurden 17,4±15,8 (3–60) Monate nach einer modifizierten Fontanoperation 13 Tunnelfenestration mit einem Amplatzer Septal Occluder (n=7), einem Rashkind Schirm (n=3), einem CardioSeal Schirm (n=1) und mit ablösbaren Coils (n=2), 3 Tunnellecks mit ablösbaren Coils (n=2) und einem Rashkind Schirm (n=1) sowie 3 intrakardiale venöse Kollateralen mit ablösbaren Coils interventionell verschlossen. Resultate Die arterielle Sauerstoffsättigung stieg von 85±4,5 (70–89)% auf 91,4±2,8 (83–95)% (p<0,001) und der mittlere Tunneldruck von 10,7±1,8 (6–14) mmHg auf 12,1±2,4 (6–16) mmHg an (p<0,001). Das errechnete Qs fiel von 5,15±2,1 (2,1–11,3) l/min/m2 auf 3,6±1,0 (1,8–5,6) l/min/m2 (p<0,001), während die zentralvenöse Sauerstoffsättigung (66,4±7,4% vs 65±7%) und der mittlere arterielle Druck (73±8mmHg vs 73±9mmHg) sich nicht änderten. Bei einem Patienten konnte ein zusätzliches Tunnelleck wegen Anstieg des Mitteldruckes im Tunnel auf über 18mmHg bei der Probeokklusion nicht verschlossen werden. Bei 2 Patienten wurde nach 16 bzw. 21 Monaten ein Restdefekt nach Coil- bzw. Schirmverschluss einer Fenestration sowie eine zusätzliche venöse Kollaterale mit Coils verschlossen. Nach einer Nachbeobachtungszeit von 42±23 (7–99) Monaten betrug die mittlere pulsoxymetrische Sauerstoffsättigung 93±2 (90–97)%. Bei 2 Patienten war echokardiographisch noch ein minimaler Restshunt sowie bei 2 weiteren Patienten eine intrapulmonale arteriovenöse Fistel nachweisbar. Bei keinem Patienten kam es zur Dislokation des Implantats, zu Thrombembolien oder zum Auftreten einer Hämolyse. Schlussfolgerung Nach modifizierten Fontanoperationen können Kommunikationen zwischen system- und pulmonalvenösem Stromgebiet je nach Morphologie und Größe mit Schirmsystemen oder ablösbaren Coils interventionell mit guten kurz- und mittelfristigen Ergebnissen verschlossen werden, um eine persistierende oder sich postoperativ entwickelnde Zyanose zu beseitigen und das Risiko einer paradoxen Embolie zu verringern.