A comparative study of three palliative surgical approaches for pulmonary atresia with ventricular septal defect / 中国胸心血管外科临床杂志

@#Objective    To compare and investigate the efficacy and differences of modified B-T shunt, central shunt and right ventricle-pulmonary artery (RV-PA) connection in the treatment of pulmonary atresia with ventricular septal defect (PA/VSD). Methods    A total of 124 children with PA/VSD underwent initial palliative repair in Shanghai Children's Medical Center from September 2014 to August 2019, including 63 males and 61 females, aged 7 days to 15 years. They were divided into in a modified B-T shunt group (55 patients), a central shunt group (22 patients) and a RV-PA connection group (47 patients). The clinical data of these children were retrospectively analyzed. Results    There were 9 early deaths after palliation, with an early mortality rate of 7.3%. The mean follow-up time was 26.5±20.3 months, with 5 patients lost to follow-up, 5 deaths during the follow-up period, and 105 survivors. The 1-year and 5-year survival rates were both 89.7%. The monthly increased Nakata index was 5.2 (–0.2, 12.3) mm2/m2, 9.2 (0.1, 23.6) mm2/m2, 6.3 (1.8, 23.3) mm2/m2 in the modified B-T shunt group, the central shunt group, and the RV-PA connection group, respectively, with no statistical difference among the three groups. The 1-year survival rate was 85.3%, 78.4%, 95.2%, and the 5-year (4-year in the central shunt group) survival rate was 85.3%, 58.8%, 95.2% in the three groups, respectively, with a statistical difference among them (P<0.05). The complete repair rate was 36.5%, 19.0% and 67.4% in the three groups, respectively, with a statistical difference among the three groups (P<0.001). Conclusion    All these three palliative surgical approaches can effectively promote pulmonary vascular development. But compared with systemic-pulmonary shunt, RV-PA connection has a lower perioperative mortality rate and can achieve a higher complete repair rate at a later stage, which is beneficial for long-term prognosis.

The application of stemic-pulmonary shunt in staged operation for tetralogy of Fallot with hypoplasia pulmonary arteries / 中华胸心血管外科杂志

Objective:The study aimed to evaluate the effect of systemic-pulmonary shunt(SPS) on the infants with tetralogy of Fallot(TOF) with hypoplasia pulmonary arteries.Methods:Among 949 infants less than three-year-old who underwent surgical intervention between January 2010 and December 2015, 853 infants underwent one-stage primary repair(group Ⅰ), whereas 96 infants underwent SPS(group Ⅱ). Among them, 546 were males and 403 were females. The average age and average weight at operation were 13 months and 9.4 kg, respectively. 20.8% infants had clinical symptoms before operation. Perioperative and follow-up parameters were assessed including age, weight, sex, transcutaneous oxygen saturation(SPO 2), preoperative clinic symptom, ratio of McGoon, NAKATA index, Z score of pulmonary annulus, left ventricular end diastolic diameter(LVEDD). Results:There were 28(3.0%) early postoperative deaths in the whole group. And no significant difference in mortality was found between group Ⅰ(2.9%) and group Ⅱ(3.1%). Compared with group Ⅰ, age and weight were significantly lower in patients in group Ⅱ[(0.95±0.56)years old vs.(1.09±0.59)years old] and[(8.6±1.8)kg vs.(9.5±2.1)kg], respectively. Patients in group Ⅱ had a lower SPO 2(0.75±0.09 vs. 0.85±0.09) and was more prone to appear hypoxic spell(30.2% vs. 19.7%) than patients in group Ⅰ. Patients in group Ⅱ had a more poor development in pulmonary artery. The ratio of McGoon, NAKATA index and Z score of pulmonary annulus in group Ⅱ were significantly less than that in group Ⅰ[1.4±0.3 vs. 1.9±0.5, (124±43)mm 2/m 2 vs.(222±88)mm 2/m 2 and-4.3±2.6 vs.-2.3±2.1], respectively. There was no difference of extubation time bewteen two groups. SPO 2 rose significantly from 0.75 to 0.83 after SPS. And after SPS, the diameter of main pulmonary artery, the ratio of McGoon, NAKATA index and Z score of pulmonary annulus increased significantly from 6.4 mm, 1.42, 126.4 mm 2/m 2 and -4.3 to 9.2 mm, 1.83, 212.6 mm 2/m 2 and -2.4, respectively. Moreover, LVEDD also increased significantly from 21.2 mm to 24.5 mm after SPS. Conclusion:SPS is safe and effective for little infants with anoxic spell and hypoplasia pulmonary arteries. SPS can improve oxygenation significantly and delay the age for radical surgery. SPS also promote the growth of the pulmonary arteries, pulmonary valve annulus and left ventricular, and it facilitates the preservation of pulmonary valve annulus and reduce the rate of transannular patching in a portion of infants.

Role of secretory protein GREM1 in systemic-to-pulmonary shunt associated pulmonary arterial hypertension / 中国胸心血管外科临床杂志

@#Objective    To explore the possibility that GREM1, a bone morphogenetic protein (BMP) antagonist, is a mechanical explanation for BMP signal suppression in congenital heart disease associated pulmonary arterial hypertension (CHD/PAH) patients. Methods    Systemic-to-pulmonary shunt induced PAH was surgically established in rats. At the postoperative 12th week, right heart catheterization and echocardiography evaluation were performed to evaluate hemodynamic indexes and morphology of right heart system. Right heart hypotrophy index and pulmonary vascular remodeling were evaluated. Changes of BMP signal pathway related proteins and GREM1 in lungs and plasma GREM1 concentration were detected. The effect of GREM1 on the proliferation and apoptosis of pulmonary arterial endothelial cells (PAECs) was also explored. Results    The hypertensive status was successfully reproduced in rats with systemic-to-pulmonary shunt model. BMP signal pathway was suppressed but GREM1 was up-regulated with no change in hypoxia inducible factor-1 in lungs exposed to systemic-to-pulmonary shunt, while this trend was reversed by systemic-to-pulmonary shunt correction (P<0.05). Immunohistochemical staining demonstrated enhanced staining of GREM1 in remodeled pulmonary arteries. In vitro experiments found that BMP signal was down-regulated but GREM1 expression and secretion were up-regulated in proliferative PAECs (P<0.05). Furthermore, BMP2 significantly inhibited PAECs proliferation and promoted PAECs apoptosis (P<0.05), which could be antagonized by GREM1. In addition, plasma level of GREM1 in rats with systemic-to-pulmonary shunt was also increased and positively correlated with pulmonary hemodynamic indexes. Conclusion    Systemic-to-pulmonary shunt induces the up-regulation of GREM1 in lungs, which promotes pulmonary vascular remodeling via antagonizing BMP cascade. These results present a new mechanical explanation for BMP pathway suppression in lungs of CHD/PAH patients.

Systemic-Pulmonary Shunt and Pulmonary Artery Banding / 日本心臓血管外科学会雑誌

Systemic-pulmonary shunt for neonate and small infant with decreased pulmonary blood flow is an important first palliative surgery as simple palliation or complex palliative open-heart surgery to affect the completeness of subsequent radical or second surgery. It is important to understand the hemodynamics according to each disease and determine the shunt design considering the “shape” and “flow rate” of the shunt. In recent years, Blalock-Taussig shunt (BT shunt) and central shunt through median sternotomy have become mainstream, however conventional BT shunt through lateral thoracotomy is still an important basic procedure which pediatric cardiac surgeons should learn. Pulmonary artery banding (PAB) or bilateral PAB is also an important palliative procedure to protect the right and left pulmonary vascular beds equally for pulmonary high-flow complex heart disease and functional single ventricle. It is essential to perform secure PAB or bilateral PAB, which leads to the next procedure smoothly.

Study of transforming growth factor-a as a diagnostic biomarker for systemic-to-pulmonary shunts induced pulmonary arterial hypertension / 中国药理学通报

Aim To determine the feasibility of transforming growth factor-a ( TGF-a ) as a diagnostic bio-marker for systemic-to-pulmonary shunts induced pulmonary arterial hypertension ( PAH ). Methods Systemic-to-pulmonary shunts induced PAH was built by combined surgery ( right pulmonary artery was ligated and a cervical shunt was established one week later). A total of 49 patients with congenital heart diseases were recruited, including 24 congenital heart disease patients without PAH(CHD) and 25 congenital heart disease patients with pulmonary arterial hypertension (CIID-PAH). Moreover, 20 healthy volunteers matched by age and sex were also included. Enzyme linked immunosorbent assay (EL1SA) was used to test TGF-a concentrations in plasma of systemic-to-pulmonary shunts induced PAH rats and CHD-PAH patients. Results ELISA results showed that TGF-a levels in plasma of systemic-to-pulmonary shunts induced PAH rats were significantly higher than those of sham operated group. Spearman correlation analysis showed that plasma TGF-a concentrations were positively associated with right ventricular systolic pressure, pulmonary arterial systolic pressure, mean pulmonary arterial pressure and right ventricular hypertrophy index. The plasma concentration of TGF-a in CHD-PAH patients was much higher than that of CHD patients and healthy vol- unteers ( CON); however, there was no significant difference between CHD group and CON group; Using 314 ng • L"1 as cutoff value of TGF-a for the diagnosis of CHD-PAH, the sensitivity, specificity and area under the cure was 0. 760, 0. 750 and 0. 895, respectively. Conclusions Plasma concentration of TGF-a increases with the progression of systemic-to-pulmonary shunt induced PAH; the level of TGF-a in plasma may be a potential biomarker for the diagnosis of systemic- to-pulmonary shunt induced PAII.

Shunt occlusion in children with congenital heart disease undergoing systemic-pulmonary shunt: an analysis of risk factors and early and mid-term results / 中华胸心血管外科杂志

Objective To determine the early and mid-term outcomes of shunt occlusion in infant and children after systemic-pulmonary shunt surgery and explore the risk factors of shunt occlusion in these patients.Methods Retrospective analysis was conducted for 295 patients with congenital heart disease after systemic pulmonary shunt surgery at Fuwai Hospital from January 2010 to August 2016.There were 183 males and 112 females with their median age 7.5 months,and mean body weight (9.2 ±4.9) kg.The major cardiac diagnosis included pulmonary atresia and ventricular septal defect(110 cases),transposition of the great arteries (67 cases),tetralogy of Fallot (38 cases) and others.The surgical procedures included central aorto-pulmonary shunt in 97 patients and modified Blalock-Taussig shunt in 198 patients.The outcomes were compared between patients with and without shunt occlusion group and risk factors were identified using univariate and multivariate logistic analysis.Results Death occurred in 25 patients in which 16％ were associated with shunt occlusion.Follow-up was complete in 92.2％ averaging 35 months(range 2 to 84 months).Multivariate logistic regression analysis identified that the central aorto-pulmonary shunt type(P =0.008),shunt size less than 4 mm(P =0.001),platelet transfusion(P =0.001) and poor compliance for aspirin antiplatelet therapy(P =0.04) were all the independent risk factors despite adjusting for other variables.While the increased postoperative oxygen saturation may be the protective factor(OR =0.93,P =0.001).Conclusion The central aortopulmonary shunt type,smaller shunt size,platelet transfusion,decreased postoperative oxygen saturation and poor compliance for ASA antiplatelet therapy were important factors of shunt occlusion in patients undergoing shunt surgery.The proper identification and management would improve the prognosis of these patients.

Effects of different doses of dexmedetomidine on cerebral oxygen saturation and pulmonary shunt fraction in patients undergoing one-lung ventilation / 天津医药

Objective To investigate the effects of different doses of dexmedetomidine on cerebral oxygen saturation and pulmonary shunt fraction in patients undergoing one-lung ventilation (OLV). Methods Sixty ASAⅠ-Ⅱpatients, aged 46-71 years, with body mass index (BMI)18-24 kg/m2 and scheduled for thoracotomy were randomly divided into three groups (n=20 each):high dose dexmedetomidine group (group D1), low dose dexmedetomidine group (group D2) and control group (group C). Dexmedetomidine 1μg/kg was infused in group D1 after anesthesia induction, and then a rate of 0.5μg·kg-1·h-1 was continuously infused. Dexmedetomidine 0.5μg/kg was infused in group D2 after anesthesia induction, and then a rate of 0.3μg · kg-1 · h-1 was continuously infused. Group C was received the equal volume of normal saline. Anesthesia was main?tained with propofol-remifentanil and intermittent iv boluses of rocuronium. Arterial and jugular venous blood samples were collected before anesthesia induction (T0), at 15 min after two-lung ventilation (T1), at 5 min (T2) and 30 min (T3) of OLV for blood gas analysis. Value of Qs/Qt was calculated and SctO2 was recorded at the same time. Results Compared with group C and group D2, Qs/Qt was decreased at T2 in group D1 (P<0.05). Qs/Qt was lower at T3 in group D1 and D2 than that of group C, and which was lower in group D1 than that of group D2 (P<0.05). In group C and group D1 a significant de?crease in SctO2 was observed at T2 and T3 compared to that at T0 and T1 (P<0.05). SctO2 was significantly higher at T2 and T3 in group D2 than that in group C and group D1 (P<0.05). Conclusion Dexmedetomidine given during OLV undergoing thoracotomy can improve oxygenation, decrease pulmonary shunt fraction and reduce the occurrence of low SctO2.

Post-operative complications and measures on complex cyanotic congenital heart diseases and pulmonary ;shunt / 中国小儿急救医学

Objective To analyze the causes and treatment methods of early complications after central systemic-pulmonary shunt in complex cyanotic congenital heart diseases.Methods Two hundred and twelve cases of central systemic-pulmonary shunt in complex cyanotic congenital heart diseases were retro-spectively analyzed in order to explore the early postoperative complications and related treatment measures. Results There were 61 cases(28.77%)of the early postoperative complications,including severe low car-diac output syndrome in 27 cases,acute pulmonary edema in 14 cases,24 h shunt pipe blockage in 12 cases, and supraventricular tachycardia in 8 cases.All patients got followed up,average for(2.49 ±1.21 )years.Af-ter the systemic-to-pulmonary artery shunts,pulmonary vascular had significant growth,8 patients(3.77%) of them who pulmonary hypoplasia were promoted by transcatheter aortopulmonary collateral vessels.At the end of the follow-up,77 patients(36.32%)achieved the standard of radical surgery.Conclusion The factors affecting surgical survival rate include:enhancement of patients cardiac function and strictly handle operation indication before operation a clear operational view;rational surgical methods;treatment of complication with-out delay;strict,intensive care and synthesized treatment.

Efficacy analysis on arterial catheter stents for pulmonary atresia with intact ventricular septum in newborns / 中华实用儿科临床杂志

Objective To compare the efficacy of arterial stenting with that of traditional B -T shunt for neo-natal pulmonary atresia with intact ventricular septum.Methods Twenty -six cases of neonatal pulmonary atresia with intact ventricular septum were treated at Children′s Hospital of Zhengzhou from December 2005 to December 201 5,aged 3 to 23 days[(8.20 ±4.80)days],and weighted 2.80 -3.88 (3.41 ±0.27)kg.Accompanied with pulmonary hypo-plasia,all these patients were combined with PDA and ASD or PFO.Before operation,the peripheral oxygen saturation was kept in 61 % -75%,averaged at 67%.Among them,1 2 cases underwent arterial catheter stenting,and 1 4 cases re-ceived B -T shunt (including modified B -T shunt and central shunt)ductus ligation.After operation,the oxygen satu-ration in these children was observed,and they were examined by echocardiography and true lateral chest X -ray,blood flow situations in stents and shunts were assessed,and follow -up examinations were conducted in 1 ,3,6 and 1 2 months postoperatively.Results Stents were successfully inserted into these 1 2 cases.After traditional pulmonary shunt,pa-tients′peripheral oxygen saturation was (82.73 ±5.59)%,compared with that of patients after arterial catheter sten-ting (86.1 8 ±3.1 9)%,there was significant difference(t =1 0.71 ,P <0.05).In pulmonary shunt group,2 cases died,1 case died of postoperative heart failure,and the other case was complicated with pulmonary infection and died of respiratory failure;in catheter stent group,1 case was complicated with postoperative supraventricular tachycardia and recovered after drug intervention.For the follow -up examinations 1 ,3,6 and 1 2 months after the operation,1 case of B -T shunt in pulmonary shunt group was improved by central shunt due to slow blood flow,less shunt volume,and ox-ygen saturation decreased to 69%.For the catheter stent group,1 case was improved by stent balloon dilatation due to declined peripheral oxygen saturation 2 months postoperatively,and after the operation,transcutaneous oxygen saturation was improved.No patients died in the catheter stent group.Conclusions In traditional B -T shunts,the operation is extensive,and complications are common,and the recovery turns slowly.Arterial catheter stenting can be used as the preferred method of treatment for one -stage surgical pulmonary atresia with intact ventricular septum to reduce the weakness feasibly and effectively.

Endovascular Repair of Recurrent Para-anastomotic Pseudoaneurysm of Occluded Potts' Shunt / 日本心臓血管外科学会雑誌

A 34-year-old female patient, who had undergone several operations relating to pulmonary atresia with ventricular septal defect (PA/VAD), was admitted to our hospital for recurrent massive hemoptysis requiring blood transfusion. She had undergone a Potts' shunt (systemic-pulmonary shunt) at age 3, and undergone intraaortic patch closure for paraanastomotic pseudoaneurysm of occluded Potts' shunt at age 28. Chest CT scan revealed an enhancement of this aneurysm surrounding ground glass like appearance of left lung lobe which indicated pulmonary bleeding. In order to avoid skeltonization of severe adhesion of mediastinal and left thoracic space due to several prior operations, endovascular repair of this pseudoaneurysm with debranching of left subclavian artery was performed. Postoperative course was uneventful and hemoptysis was disappeared. She was discharged at 8 postoperative day.

Effect of nasal continuous positive airway pressure and Bi-phase positive airway pressure on oxygen exchange indexes in newborns with mild to moderate neonatal respiratory distress syndrome / 中华实用儿科临床杂志

Objective To observe the effect of nasal continuous positive airway pressure (nCPAP) and Biphase positive airway pressure (BIPAP) on the oxygen exchange indexes in newborns with mild to moderate neonatal respiratory distress syndrome (NRDS).Methods After infusion of pulmonary suffactant into lung bilaterally,116 cases with mild to moderate NRDS were treated with nCPAP(nCPAP group,n =75) and BIPAP(BIPAP group,n =41) respectively.Oxygen exchange indexes including arterial partial pressure of oxygen [pa (O2)],modified oxygenation index (P/F),alveolar-arterial oxygen tension difference (A-aDO2),respiratory index (RI),artery/alveolar oxygen partial pressure ratio[pa (O2)/pA (O2)] and pulmonary shunt fraction (Qs/Qt) were measured or calculated at 0 (before treatment),2,8 and 24 h after being treated with nCPAP or BIPAP.Results Six different oxygen exchange indexes of Pa (O2),P/F,A-aDO2,RI,pa (O2)/pA(O2) and Qs/Qt in both groups didn't show statistical significance at 0 hour:pa(O2) (kPa) (6.1 ±0.6 vs 6.1 ±0.6,t =0.11,P ＞0.05),P/F(kPa) (15.59 ± 1.45 vs 15.71 ± 1.45,t =1.59,P ＞ 0.05),A-aDO2(kPa)(6.04 ±0.64 vs 6.24 ±0.69,t =1.59,P ＞0.05),RI(6.0 ± 1.0 vs 6.2 ± 1.1,t =1.35,P ＞ 0.05),p,(O2)/pA(O2) (0.24 ±0.02 vs 0.25 ±0.03,t =1.63,P ＞0.05)and Qs/Qt(％)(11.9±1.6 vs 11.6 ± 1.6,t =1.10,P ＞ 0.05).A-aDO2,RI,pa (O2)/pA (O2) and Qs/Qt of BIPAP group had more improvement than those of nCPAP at 2 and 24 h,and oxygen exchange indexes at 2 h of BIPAP group and those of nCPAP group were:A-aDO2 (kPa) (3.83 ±0.49 vs 4.24 ± 0.67,t =18.26,P ＜ 0.05),RI(2.7 ± 0.5 vs 3.3 ±0.7,t =20.59,P ＜ 0.05),p,(O2)/pA(O2) (0.35 ±0.03 vs 0.32 ±0.04,t =15.35,P ＜0.05) and Qs/Qt(％) (8.8 ± 1.6 vs 9.8 ±2.0,t =7.68,P ＜ 0.05) ; 24 h indexes in BIPAP group and the indexes of nCPAP group were:A-aDO2 (kPa) (2.29 ± 1.19 vs 3.07 ± 1.67,t=18.43,P ＜0.05),RI(1.4 ±1.4 vs 2.3 ± 1.6,t=25.02,P ＜0.05),pa(O2)/pA(O2) (0.49 ±0.10 vs 0.42 ±0.11,t =14.96,P ＜0.05) and Qs/Qt(％) (6.5 ±3.0 vs 8.5 ±4.4,t =9.59,P ＜0.05).pa(O2) and P/F of both groups didn't show statistical difference significantly [Pa (O2)(kPa) (12.6 ± 1.0 vs 12.7 ± 1.0,t =3.76,P ＞ 0.05),P/F(28.49 ± 3.17 vs 31.85 ± 2.85,t =3.76.P ＞ 0.05)].Nineteen cases in nCPAP group needed invasive mechanical ventilation,and only 3 cases in BIPAP group underwent the procedure;there was a significant difference (x2 =4.01,P ＜ 0.05).Oxygen exchange indexes in both groups were gradually improved from 2 h,8 h to 24 h,but 22 deteriorated cases showed no improvement,who underwent invasive ventilation.Positive end-expiratory pressure (PEEP) positive airway pressure (EPAP) was (0.42 ± 0.19) kPa in BIPAP group,and (0.56 ± 0.23) kPa in nCPAP group (t =3.45,P ＜ 0.01).Conclusions 1.BIPAP and nCPAP can improve the ability of oxygen exchange in newborns with mild to moderate NRDS,but BIPAP is more effective than nCPAP,which can shorten the possibility of invasive ventilation and reduce the PEEP level.2.p,(O2),P/F,pa (O2)/pA (O2) and RI may be more suitable for evaluating oxygen exchange for neonatal NRDS.

Novel Partial Anomalous Pulmonary Venous Connection Associated with Brain Abscess

BACKGROUND: Right-to-left vascular shunts are associated with brain abscess. CASE REPORT: We present a 47-year-old female with a cryptogenic left thalamic abscess on which Streptococcus mitis grew upon aspiration. Computed tomography of the chest with contrast agent revealed an anomalous connection between the left superior pulmonary and brachiocephalic veins. A right-to-left shunt was confirmed in a transthoracic echocardiogram study in which bubbles were injected into the left arm; this shunt had not previously been noted upon right-arm injection. CONCLUSIONS: We recommend aggressive evaluation for right-to-left shunts in patients who present with cryptogenic brain abscesses. In addition to imaging, this should include a bubble-based study with left-arm saline injection.

Síndrome hepatopulmonar en pediatría: presentación de un caso y revisión de la literatura / Hepatopulmonary syndrome in children: a case report and literature review

Hepatopulmonary syndrome is a rare disease that affects patients of any age with acute or chronic liver disease. The prevalence and its outcome in children are unknown. Clinically are common shortness of breath, orthodeoxia and platypnoea, together with cyanosis, digital clubbing and spider naevi. Its diagnosis is based in a clinical triad characterized by liver disease with or whithout portal hypertension, hypoxemia and intrapulmonary vascular dilatation leading to right and left shunts, demonstrated by echocardiography with contrast, the perfusion lung scanning using technetium-labelled macro-aggregates albumin estimate the shunt fraction. Pulmonary angiography is useful to demonstrate macroscopic arteriovenous communications. The orthotopic liver transplantation is the only efficacy treatment in patients without several gas exchange abnormality.

El síndrome hepatopulmonar es una enfermedad poco frecuente que afecta a pacientes de cualquier edad con enfermedad hepática aguda o crónica. La prevalencia y la evolución en pediatría son desconocidas. Clínicamente es común encontrar disnea de esfuerzo, platipnea y ortodeoxia, además de cianosis, acropaquia y nevi aracniformes. El diagnóstico está basado en una tríada clínica caracterizada por una enfermedad hepática con o sin hipertensión portal, hipoxemia y vasodilatación intrapulmonar que conduce a un shunt de derecha a izquierda, demostrado mediante ecocardiografía con contraste y cintigrafía de perfusión pulmonar con albúmina macroagregada marcada contecnesio-99m que permite la estimación de la fracción del shunt. La angiografía pulmonar es útil para demostrar comunicaciones arteriovenosas macroscópicas. El trasplante hepático es el único tratamiento demostradamente eficaz, excepto en aquellos pacientes con un trastorno ventilatorio grave, por su mayor morbimortalidad.

Bidirectional Cavopulmonary Shunt for Isolated Right Ventricular Endomyocardial Fibrosis / 대한흉부외과학회지

A 44-year-old man had been admitted for dyspnea on exertion and abdominal distension. The echocardiography revealed abnormal mass in right atrium and tricuspid valve stenosis with right ventricular obliteration. The operation was performed with mass removal, enlargement of tricuspid valve opening, and right ventricular endocardiectomy. And then, atrial septal defect was made due to inadequate right ventricular volume. The patient's symptom was improved and he discharged without events. The endomyocardial fibrosis was diagnosed with microscopic examination. Eighteen months later, the patient was readmitted due to aggravated dyspnea and cyanosis. The right ventricular obliteration was progressed and pulmonary blood flow was severely decreased in follow up echocardiography. Palliative bidirectional cavo-pulmonary shunt was performed due to functional single ventricle. The dyspnea and cyanosis was markedly improved. Bidirectional cavo-pulmonary shunt for advanced and isolated right ventricular endomyocardial fibrosis provided effective palliation at early postoperative period, However, long-term follow up is mandatory.

Anesthetic Management for Resection of a Pulmonary Arteriovenous Malformation / 대한마취과학회지

Pulmonary arteriovenous malformation (PAVM) is an uncommon congenital anomaly. As PAVM is a direct communication between branches of the pulmonary artery and vein, severe hypoxemia, paradoxical embolism, and massive hemorrhage can result. We present a 39-year-old woman with severe hypoxemia after the induction of one lung ventilation (OLV) for resection of a PAVM in her right lower lobe. We analyzed the cause of hypoxemia by an arterial blood gas analysis and estimated shunt equation. The preoperative value of an intrapulmonary shunt (Qs/Qt) was 15%. However, after the OLV, the values of Qs/Qt increased to 48% with 92.1% arterial oxygen saturation (SaO2). After the resection of PAVM, the value of Qs/Qt decreased to 36% during the OLV, and postoperative value of Qs/Qt and SaO2 were in the normal range. These findings represent that PAVM patients are prone to severe hypoxemia and an abnormally high Qs/Qt, which might be induced by the increase of pulmonary vascular resistance during OLV.

Clinical study of staged repair for tetralogy of Failot with pulmonary artery hypoplasty / 中华胸心血管外科杂志

Objective To evaluate the effect of staged repair for tetralogy of Fallot (TOF) associated with pulmonary artery hy- poplasty.Methods From June 1996 to June 2006,37 patients with TOF were operated on.There were 26 males and 11 females. Their age was 5 months to 17 years(mean 3.6 years) and weight was 4.6～38.0 kg.All patients were diagnosed as TOF with pulmo- nary artery hypoplasty by cardiac catheterization.The mean pre-operative arterial saturation of the patients was (68.2?6.5) %,Mc- Coon ratio was 0.95?0.26 (0.81～1.17) and Nakata index was 82.7?21.6(71.6～97.5) mm~3/m~2.At the time of the first surgi- cal procedure,17 patients underwent central aortopulmonary shunt,13 patients received modified Blalock-Taussig shunt in the left side and 7 patients had modified Blalock-Taussig shunt in the right side.Results There were no easly operative deaths and no late deaths after the first stage repair.Pleural effusion after shunt occurred in 5 patients.The mean arterial saturation was significantly increased to (91.3?10.4) %,P

Effect of Changes in Fraction of Inspired Oxygen(F1O2) on Pulmonary Shunt Ratio in Respiratory Intensive Care / 대한마취과학회지

For the purpose of recommending appropriate fraction of inspired oxygen(F1O2) for long term mechanical ventilation support by evaluating the effect of changes in F1O2 on pulmonary shunt ratio in respiratory intensive care, the effects of various F1O2 on pulmonary shunt ratio were investigated and analysed with 20 pediatric intensive care patients who had received open heart surgeries for their congenital heart diseases in the department of Pediatrics, Seoul National University Hospital. The results were as follows ; 1. Through the whole process of control of mechanical ventilation the arterial CO2 tension(PaCO2) showed hyperventilation with the average of 31-34mmHg and pH showed pure respiratory alkalosis with the average of 7.45-7.51. As the fraction of inspired oxygen decreased, the arterial oxygen tension decreased. 2. Inspite of the change of F1O2, analysis of central venous blood gas showed no significant change. 3. The alveolar-arterial oxygen tension difference(AaDO2) was positively related to F1O2. 4. The pulmonary shunt ratio(Q's/Q'T) showed no significant difference in the F1O2 range of 0.6 to 1.0. But the Q's/Q'T in the F1O2 range of 0.3 to 0.4 decreased significantly comparing to that of 0.6 to 1.0 F1O2 range. Linear regression analysis of Q's/Q'T showed that the Q's/Q'T was positively related to F1O2 and AaDO2, respectively.

Effects of positive end-expiratory pressure on pulmonary shunt during geneal anesthesia and after the operation / 中华麻醉学杂志

The effects of positive end-expiratory pressure (PEEP) on pulmonary shunt were studied during gen- eral anesthesia and postoperative period.Twenty cholecystectomy patients were randomly divided into experiment group (group P) and control group (group Z). PEEP and ZEEP were used separately after induction. Artery blood and mixed blood from the right ventricle were taken for blood gas analysis and determine the amount of pulmonary shunting before anesthesia. half and hour, one and half an hour and two and half an hour after anesthesia and one hour after the operation.The results showed that shunt in group P decreased gradually during general anesthesia and returned to the level of preoperation at an hour after operation. Shunt in group Z was increased continually and the level was significantly higher than preoperation an hour after operation. Shunt between two groups was significant difference (P

Derivaçäo sistêmico-pulmonar em cardiopatias congênitas com hipofluxo pulmonar: análise crítica da técnica cirúrgica e resultados imediatos / Systemic-pulmonary shunts in congenital heart diseases with decreased pulmonary blood flow: critical analysis of surgical technics and immediate results

Objetivo - Avaliar o resultado pós-operatório imediato de crianças portadoras de cardiopatias congênitas com hipofluxo pulmonar, submetidas à derivaçäo sistêmico-pulmonar. Casuística e Métodos - Sessenta e quatro pacientes, 30 (46,8%) do sexo masculino, com idades entre 1 dia e 17 anos, divididos em: grupo I, 13 (20,3%) pacientes submetidos à operaçäo de Blalock-Taussig (B-T) clássica; grupo II, 46 (71,8%) pacientes submetidos à B-T modificada, sendo usados 2 tipos de enxertos, polytetrafluoroetylene (PTFE) em 34 casos e veia umbilical em 12; grupo III, em 5 (7,8%) pacientes submetidos à derivaçäo central com emprego de 3 tipos de enxertos, PTFE em 3, veia umbilical em 1 e artéria mamária bovina em 1. Os procedimentos associados foram ligadura do canal arterial persistente em 2 casos, ligadura de artérias brônquicas em 3, valvotomia pulmonar em 3, ampliaçäo de via de saída do ventrículo direito em 3 e apliaçäo de estenose de artéria pulmonar em outros 2 casos. Resultados - No grupo I houve 4 (30,7%) casos com obstruçäo da derivaçäo, tendo sido 2 reoperados, com mortalidade de 30,7%. No grupo II houve 4 (8,6%) casos com obstruçäo e 2 reoperaçöes com mortalidade de 15,2% (7 casos) e no grupo III houve 1 (20%) de obstruçäo e mortalidade de 80% (4 casos). A mortalidade relacionada exclusivamente à derivaçäo foi de 15,3%, 8,6% e 40% respectivamente. Conclusäo - A operaçäo de B-T modificada, realizada com maior freqüência, apresentou menor índice de obstruçäo e menor mortalidade, sendo recomendada como primeira escolha

Tricuspid atresia associated with double orifice mitral valve and coronary sinus septal defect

No abstract available.