Pulmonary Hemodynamic Changes with Nitric Oxide or Oxygen in a Patient with Asplenia, Single Right Ventricle, and Total Anomalous Pulmonary Venous Connection after Fontan Procedure

Asplenia syndrome is frequently complicated by a total anomalous pulmonary venous connection. Pulmonary venous obstruction, following total anomalous pulmonary venous connection surgery, is one of the risk factors for morbidity and mortality. In some patients, the pulmonary vasculature is abnormal even in the absence of clinical evidence of pulmonary venous obstruction. We hypothesized that a change in the pulmonary hemodynamics could indicate the abnormality of pulmonary vein in a patient with asplenia, single right ventricle, and total anomalous pulmonary venous connection, following Fontan procedure. Here, we present a case of asplenia, single right ventricle, total anomalous pulmonary venous connection, and right pulmonary venous obstruction in which evidence of a potential left pulmonary venous obstruction was obtained following the administration of inhaled nitric oxide and oxygen.

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Total Anomalous Pulmonary Venous Connection with Severe Pulmonary Venous Obstruction

Circulation ◽

10.1161/01.cir.25.6.916 ◽

1962 ◽

Vol 25 (6) ◽

pp. 916-928 ◽

Cited By ~ 54

Author(s):

ALOIS R. HASTREITER ◽

MILTON H. PAUL ◽

MARIAN E. MOLTHAN ◽

ROBERT A. MILLER

Keyword(s):

Pulmonary Venous Obstruction ◽

Venous Obstruction ◽

Anomalous Pulmonary Venous Connection

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Total anomalous pulmonary venous connection

ESC CardioMed ◽

10.1093/med/9780198784906.003.0196 ◽

2018 ◽

pp. 831-834

Author(s):

Marietta Charakida ◽

John Deanfield

Keyword(s):

Long Term Results ◽

Pulmonary Venous Obstruction ◽

Venous Obstruction ◽

Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection is repaired in childhood and the long-term results are usually excellent. Recent surgical advances have reduced further the rate of recurrent pulmonary venous obstruction.

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Surgical treatment of total anomalous pulmonary venous connection —Clinical aspects of pulmonary venous obstruction—

The Japanese Journal of Thoracic and Cardiovascular Surgery ◽

10.1007/bf03217887 ◽

1998 ◽

Vol 46 (11) ◽

pp. 1126-1132

Author(s):

Katsunori Yoshihara ◽

Tsukasa Ozawa ◽

Hiroshi Sakuragawa ◽

Takeshirou Fujii ◽

Noritsugu Shiono ◽

...

Keyword(s):

Surgical Treatment ◽

Pulmonary Venous Obstruction ◽

Clinical Aspects ◽

Venous Obstruction ◽

Anomalous Pulmonary Venous Connection

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In situ pericardium repair of pulmonary venous obstruction after repair of total anomalous pulmonary venous connection

The Japanese Journal of Thoracic and Cardiovascular Surgery ◽

10.1007/bf03032627 ◽

2002 ◽

Vol 50 (8) ◽

pp. 338-340 ◽

Cited By ~ 1

Author(s):

Hiroyuki Nishi ◽

Kyoichi Nishigaki ◽

Youichi Kume ◽

Katsuhiko Miyamoto

Keyword(s):

Pulmonary Venous Obstruction ◽

Venous Obstruction ◽

Anomalous Pulmonary Venous Connection

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Neonatal repair of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome: a case report

Journal of Cardiothoracic Surgery ◽

10.1186/s13019-021-01722-5 ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

Takahiro Ito ◽

Ikuo Hagino ◽

Mitsuru Aoki ◽

Kentaro Umezu ◽

Tomohiro Saito ◽

...

Keyword(s):

Computed Tomography ◽

Pulmonary Veins ◽

Median Sternotomy ◽

Bronchial Tree ◽

Pulmonary Venous Obstruction ◽

Goldenhar Syndrome ◽

Venous Obstruction ◽

Vertical Vein ◽

Lung Agenesis ◽

Anomalous Pulmonary Venous Connection

Abstract Background Total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome is extremely rare. Case presentation We present a case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome in a patient who was diagnosed based on transthoracic echocardiography and computed tomography. We observed complete absence of the lung, the bronchial tree, and vascular structures on the right side, with abnormal drainage of the left pulmonary veins into the innominate vein. The patient showed clear clinical evidence of pulmonary venous obstruction and underwent surgery 3 days after birth. The pulmonary venous chamber containing the vertical vein was anastomosed to the left atrium using 7–0 PDS running sutures via a median sternotomy. Echocardiography and computed tomography performed 1 year postoperatively revealed no pulmonary venous obstruction. Conclusion We report a rare case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome, which was successfully repaired 3 days after birth. A median sternotomy is a safe and effective approach for surgical repair of congenital heart disease with unilateral lung agenesis. Repair of the supra cardiac total anomalous pulmonary connection using the vertical vein is feasible in patients with a small pulmonary venous chamber.

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Unaltered vasoconstrictor responsiveness after iNOS inhibition in lungs from chronically hypoxic rats

AJP Lung Cellular and Molecular Physiology ◽

10.1152/ajplung.1999.276.1.l122 ◽

1999 ◽

Vol 276 (1) ◽

pp. L122-L130 ◽

Cited By ~ 16

Author(s):

Thomas C. Resta ◽

Theresa L. O’Donaughy ◽

Scott Earley ◽

Louis G. Chicoine ◽

Benjimen R. Walker

Keyword(s):

Pulmonary Vasculature ◽

Hypoxic Exposure ◽

Inos Mrna ◽

Mrna Levels ◽

Pulmonary Hemodynamics ◽

Pulmonary Hemodynamic ◽

Ribonuclease Protection ◽

Oxide Synthase ◽

Inos Inhibition

Previous studies suggest that inducible (i) nitric oxide synthase (NOS) expression within the pulmonary vasculature is increased in rats with chronic hypoxia (CH)-induced pulmonary hypertension. We therefore hypothesized that enhanced iNOS expression associated with CH causes attenuated pulmonary vasoconstrictor responsiveness. To test this hypothesis, we examined the effect of selective iNOS blockade withl- N 6-(1-iminoethyl)lysine dihydrochloride (l-NIL) and nonselective NOS inhibition with N ω-nitro-l-arginine (l-NNA) on vasoconstrictor responses to U-46619 in isolated saline-perfused lungs from both control and CH (4 wk at 380 mmHg) rats. We additionally measured pulmonary hemodynamic responses tol-NIL in conscious CH rats (fraction of inspired O2 = 0.12). Finally, iNOS mRNA levels were assessed in lungs from each group of rats using ribonuclease protection assays. Despite a significant increase in iNOS mRNA expression after exposure to CH, responses to U-46619 were unaltered by l-NIL but augmented by l-NNA in lungs from both control and CH rats. Pulmonary hemodynamics were similarly unaltered by l-NIL in conscious CH rats. We conclude that iNOS does not modulate pulmonary vasoconstrictor responsiveness after long-term hypoxic exposure.

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